Weinberg’s Color Atlas of Pediatric Dermatology [5th Edition] 9780071792257

Table of contents :
Section 1: Benign Neonatal Dermatoses
Section 2: Milia, Miliaria, and Pustular and Acneiform Disorders
Section 3: Bacterial Infections
Section 4: Spirochetal, Protozoal, and Mycobacterial Diseases
Section 5: Viral and Rickettsial Diseases
Section 6: Fungal Infections
Section 7: Bites and Infestations
Section 8: Allergic, Eczematous, Irritant, and Light-Related Dermatoses
Section 9: Papulosquamous, Lichenoid, and Perforating Disorders
Section 10: Nutritional and Metabolic Disorders
Section 11: Genodermatoses
Section 12: Ichthyoses and Disorders of Keratinization
Section 13: Urticarial, Purpuric, and Vascular Reactions
Section 14: Bullous, Pustular, and Ulcerating Diseases
Section 15: Cutaneous Manifestations of Systemic Disease
Section 16: Cutaneous Manifestations of HIV Infection
Section 17: Disorders of the Dermis (Infiltrates, Atrophies, and Nodules)
Section 18: Drug Eruptions
Section 19: Panniculopathies
Section 20: Vascular and Lymphatic Dysplasias
Section 21: Neoplastic Diseases
Section 22: Adnexal Dysplasias
Section 23: Benign and Malignant Pigmented Lesions
Section 24: Miscellaneous Pigmentary Disorders
Section 25: Artifacts
Section 26: Disorders of Nails and Hair
Section 27: Miscellaneous Anomalies

Citation preview

We in b e rg ’s

C O LO R A T LA S O F

PEDIATRIC DERMATOLOGY

N O T IC E Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. T e authors and the publisher o this work have checked with sources believed to be reliable in their e orts to provide in ormation that is complete and generally in accord with the standards accepted at the time o publication. However, in view o the possibility o human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication o this work warrants that the in ormation contained herein is in every respect accurate or complete, and they disclaim all responsibility or any errors or omissions or or the results obtained rom use o the in ormation contained in this work. Readers are encouraged to con rm the in ormation contained herein with other sources. For example and in particular, readers are advised to check the product in ormation sheet included in the package o each drug they plan to administer to be certain that the in ormation contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications or administration. T is recommendation is o particular importance in connection with new or in requently used drugs.

We in b e rg ’s

C O LO R A T LA S O F

PEDIATRIC DERMATOLOGY F IF TH E D ITIO N

N E IL S . P R O S E , M D , FA A P Profe s s or of De rm atology and Pe diatrics Duke Unive rs ity Me dical Ce nte r Durham , North Carolina

LE O N A R D KR IS TA L, M D , FA A P Clinical As s is tant Profe s s or of De rm atology and Pe diatrics Stony Brook Unive rs ity Stony Brook, New York

Ne w York Chicago San Francis co Athe ns London Madrid Milan Ne w De lhi Singapore Sydne y Toronto

Me xico City

Weinberg’s Color Atlas o Pediatric Dermatology, Fi h Edition Copyright © 2017 by McGraw-Hill Education. All rights reserved. Printed in China. Except as permitted under the United States Copyright Act o 1976, no part o this publication may be reproduced or distributed in any orm or by any means, or stored in a data base or retrieval system, without the prior written permission o the publisher. Previous editions published as Color Atlas of Pediatric Dermatology copyright © 2008, 1998, 1990, 1975 by T e McGraw-Hill Companies, Inc. 1 2 3 4 5 6 7 8 9 DSS 21 20 19 18 17 16 ISBN 978-0-07-179225-7 MHID 0-07-179225-2 T is book was set in Minion by Cenveo Publisher Services. T e editors were Karen G. Edmonson and Robert Pancotti. T e production supervisor was Catherine H. Saggese. Project management was provided by Kritika Kaushik, Cenveo Publisher Services. T e text designer was Eve Siegel; the cover designer was Anthony Landi. RR Donnelley was the printer and binder.

Library of Congress Cataloging-in-Publication Data Names: Prose, Neil S., author. | Kristal, Leonard, author. | Preceded by (work): Weinberg, Samuel, 1926-2007 Color atlas o pediatric dermatology. itle: Weinberg’s color atlas o pediatric dermatology / Neil S. Prose, Leonard Kristal. Other titles: Color atlas o pediatric dermatology Description: Fi h edition. | New York : McGraw-Hill Education, [2017] | Preceded by Color atlas o pediatric dermatology / Samuel Weinberg, Neil S. Prose, Leonard Kristal. 4th ed. c2008. | Includes bibliographical re erences and index. Identi ers: LCCN 2016014420| ISBN 9780071792257 (hardcover) | ISBN 0071792252 (hardcover) Subjects: | MESH: Skin Diseases | Child | In ant | Atlases Classi cation: LCC RJ511 | NLM WS 17 | DDC 618.92/5—dc23 LC record available at https://lccn.loc.gov/2016014420 McGraw-Hill Education books are available at special quantity discounts to use as premiums and sales promotions or or use in corporate training programs. o contact a representative, please visit the Contact Us pages at www. mhpro essional.com.

T is book is dedicated to the memory of Dr. Samuel Weinberg.

Dr. Samuel Weinberg

Dr. Weinberg graduated rom the Chicago Medical School in 1948. A er completing an internship and residency in pediatrics, he went into the private practice o pediatrics. T rough his personal experience, Dr. Weinberg came to understand the importance o skin disease in children and how little was known about its diagnosis and treatment. A er several years o pediatric practice, he began training in dermatology at the Skin & Cancer Hospital, a part o the New York University Postgraduate Medical School. When his dermatology training was completed, Dr. Weinberg began a private pediatric dermatology practice in Long Island, New York. At Bellevue Hospital in 1962, he ounded one o the rst clinics in the United States devoted to the care o childhood skin disease. He served there or many years as the Chie o Pediatric Dermatology and remained active as a Clinical Pro essor at the New York University School o Medicine until the time o his death. In the 1970s, Dr. Weinberg helped to ound the Society or Pediatric Dermatology. His dedication to pediatric dermatology led him to coauthor the rst edition o the Color Atlas of Pediatric Dermatology in 1975. In 2007, shortly a er completing work on the ourth edition o our book, Dr. Weinberg passed away. He will always be remembered as a great teacher, a wonder ul mentor, an inspiration to the hundreds o residents he helped to train, and a physician with unparalleled diagnostic acumen and devotion to his young patients. Dr. Weinberg was a great riend to those o us who were privileged to know him. Neil S. Prose Leonard Kristal

v

CREDITS FOR PHOTOGRAPHS

We would like to thank all o the contributors to this Atlas during the past 40 years. Arturo Aballi A. Bernard Ackerman J. O’D. Alexander Howard Balbi William G. Ballinger Charles S. Bara Robert Baron Alexander G. Bearn Jerrold M. Becker Bernard W. Berger Kassahun Bilcha Eugene L. Bodian Alanna Bree Roman Bron enbrener Martin H. Brownstein William Burke Hector Caceres-Rios Philip Charney Platon J. Collipp Maurice J. Costello Vincent Derbes Ncoza Dlova Anthony N. Domonkos Carola Duran-McKinster Lawrence Eichen eld Leon Eisenbud Nancy B. Esterly Robert P. Feinstein Ilona J. Frieden

Alexander A. Fisher Robert W. Goltz Bernardo Gontijo Ralph W. Grover Paul Honig Jonathan Horwitz Sidney Hurwitz Kathleen L. Hussey Jose E. Jelinek S. Wayne Klein Irwin H. Krasna Jose Kriner eresita A. Laude Lawrence Leiblich Chester M. Lessenden, Jr. Moise Levy Luther B. Lowe Anthony Mancini Andrew Margileth Patricia M. Mauro Diana McShane John McSorley Denise Metry Dean S. Morrell Anisa Mosam Elise Olsen Seth J. Orlow Lamar S. Osment Greg Puglisi

John R. . Reeves Perry Robins Victor orres Rodriguez Leah Ronald Avron Ross James P. Rotch ord Ramon Ruiz-Maldonado Ana Saenz-Cantele Wiley M. Sams Arthur Sawitsky Lawrence A. Schachner Keith M. Schneider Edward Shapiro Meyer H. Slatkin Roy Stephens Conrad Stritzler Ronald Stritzler Virginia Sybert Joel A. eisch Louis obin Donald Waldor William A. Welton Zelma Wessely David A. Whiting Mary Williams Constance Y. Wong Albert Yan Alex W. Young, Jr. Erwin Zimmerman

Department o Dermatology, College o Physicians and Surgeons, Columbia University New York University School o Medicine (Skin and Cancer Unit) permitted use o photographs or the ollowing gures: 2-12, 3-40, 4-7, 4-14, 4-30, 6-44, 7-2, 8-10, 8-32, 8-42, 8-43, 8-46, 9-6, 9-11, 11-3, 12-46, 12-47, 12-84, 12-88, 13-4, 13-14, 13-20, 14-8, 14-28, 14-29, 14-42, 14-77, 15-11, 15-52, 15-56, 15-57, 16-6, 17-26, 23-36, 24-9, 26-2, 28-6, 29-5, 29-45, 30-17, 30-18. T e ollowing gures have been used with permission: Figure 14-61: Listernick RH, Charrow J. T e neuro bromatoses. In: Wol K, Goldsmith LA, Katz SI et al (eds). Fitzpatrick’s Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. Figures 15-59 and 15-60: Frieden IJ, Esterly NB. Selected genodermatoses in in ants and children. Clin Dermatol. 1985 Jan-Mar;3(1):14-32. © Elsevier. Figures 18-1 and 18-8: Prose NS. HIV in ection in children. J Am Acad Dermatol. 1990 Jun;22:1223-31. © Elsevier. Figure 18-3:Prose NS, Mendez H, Meniko H, Miller HJ. Pediatr Dermatol. 1987 Aug;4(2):67-74. © John Wiley and Sons. Figures 18-4 and 18-7: Prose NS. Human immunode ciency virus in ection in childhood: T e disease and its cutaneous mani estations. Adv Dermatol. 1990;5:113-30. © Elsevier. Figures 29-36 and 29-37: Whiting DA. Hair sha de ects. In: Olsen EA (ed). Disorders of Hair Growth: Diagnosis and reatment. 2nd ed. New York: McGraw-Hill; 2003:138-39.

vi

CONTENTS

Cre dits o r Pho to g raphs Page vi Pre ace Page xix S e ctio n 1 Be nig n Ne o natal De rm ato s e s Page 1 Figures 1-1–1-4 1-5–1-8

Erythema toxicum neonatorum ransient neonatal pustular melanosis

S e ctio n 2 Milia, Miliaria, and Pus tular and Acne i o rm Dis o rde rs Page 5 Figures 2-1, 2-2 2-3 2-4–2-7 2-8, 2-9 2-10 2-11, 2-12 2-13, 2-14 2-15–2-17 2-18 2-19–2-24 2-25, 2-26 2-27 2-28, 2-29 2-30 2-31–2-33 2-34–2-36 2-37 2-38, 2-39 2-40, 2-41 2-42 2-43, 2-44

Milia Eosinophilic pustular olliculitis o in ancy In antile acropustulosis Miliaria crystallina Miliaria rubra (prickly heat) Fox-Fordyce disease (apocrine miliaria) Neonatal cephalic pustulosis Neonatal and in antile acne In antile cystic acne Acne vulgaris Cystic acne Acne conglobata Scarring ollowing acne Acne and precocious puberty rom a pinealoma Steroid acne (dexamethasone) Hidradenitis suppurativa Rosacea Periori cial dermatitis Periori cial granulomatous dermatitis Periori cial dermatitis Dissecting cellulitis o the scalp

S e ctio n 3 Bacte rial In e ctio ns Page 19 Figures 3-1, 3-2 3-3, 3-4 3-5 3-6 3-7–3-11 3-12 3-13–3-15 3-16 3-17, 3-18 3-19–3-22 3-23 3-24 3-25–3-30 3-31 3-32 3-33, 3-34 3-35, 3-36 3-37, 3-38 3-39

Impetigo Bullous impetigo Impetiginization Ecthyma Staphylococcal scalded skin syndrome Chancri orm pyoderma Folliculitis Hot tub olliculitis Furuncle Streptococcal intertrigo Perianal streptococcal disease Blistering distal dactylitis Scarlet ever Erysipelas Invasive group A streptococcal disease Cat-scratch disease Erythrasma Verruga peruana (Carrion disease) Pitted keratolysis

v ii

v iii

Conte nts

3-40 3-41, 3-42 3-43, 3-44 3-45, 3-46

Actinomycosis Cutaneous e ects o Pseudomonas sepsis Cutaneous e ects o meningococcemia Cutaneous e ects o gonococcemia

S e ctio n 4 S piro che tal, Pro to zo al, Myco bacte rial, and Ricke tts ial Dis e as e s Pag e 33 Figures 4-1, 4-2 4-3–4-6 4-7 4-8–4-11 4-12, 4-13 4-14, 4-15 4-16, 4-17 4-18 4-19 4-20, 4-21 4-22, 4-23 4-24 4-25 4-26–4-28 4-29 4-30, 4-31 4-32, 4-33 4-34, 4-35

Congenital syphilis Acquired syphilis Yaws Erythema migrans (Lyme disease) Lepromatous leprosy uberculoid leprosy Dimorphous leprosy Primary complex o tuberculosis in the skin Scro uloderma Lupus vulgaris uberculosis cutis verrucosa Lichen scro ulosorum Papulonecrotic tuberculid In ection with atypical mycobacteria Amebiasis cutis Leishmaniasis Rickettsialpox Rocky Mountain spotted ever

S e ctio n 5 Viral Dis e as e s Page 43 Figures 5-1–5-8 5-9, 5-10 5-11, 5-12 5-13–5-20 5-21, 5-22 5-23, 5-24 5-25, 5-26 5-27, 5-28 5-29 5-30, 5-31 5-32 5-33 5-34 5-35 5-36–5-38 5-39 5-40, 5-41 5-42–5-48 5-49 5-50, 5-51 5-52–5-55 5-56–5-58 5-59–5-62 5-63–5-65 5-66 5-67, 5-68 5-69, 5-70 5-71–5-74 5-75 5-76 5-77, 5-78

Molluscum contagiosum Molluscum contagiosum dermatitis Molluscum contagiosum id reaction Verruca vulgaris Plantar warts Fili orm warts Verruca plana Condyloma acuminatum Epidermodysplasia verruci ormis Neonatal herpes simplex Herpes simplex Herpetic whitlow Herpetic gingivostomatis Recurrent herpes simplex Herpes simplex, recurrent Genital herpes simplex Eczema herpeticum (Kaposi varicelli orm eruption) Varicella Congenital varicella syndrome Neonatal varicella Herpes zoster Hand- oot-mouth disease Atypical hand- oot-mouth disease Complications o vaccinia Eczema vaccinatum Exanthem subitum (roseola) Unilateral laterothoracic exanthem Papular acrodermatitis o childhood (Gianotti-Crosti syndrome) Congenital cytomegalovirus in ection Congenital rubella Rubella

ix

Conte nts

5-79–5-81 5-82 5-83–5-86 5-87, 5-88

Measles Atypical measles Erythema in ectiosum ( h disease) Papular purpuric sock and glove syndrome

S e ctio n 6 S upe rf cial Fungal In e ctio ns Pag e 67 Figures 6-1–6-3 6-4 6-5, 6-6 6-7–6-10 6-11, 6-12 6-13, 6-14 6-15–6-19 6-20 6-21 6-22 6-23, 6-24 6-25, 6-26 6-27–6-30 6-31, 6-32 6-33 6-34 6-35–6-40 6-41, 6-42 6-43 6-44 6-45–6-48 6-49, 6-50 6-51, 6-52

inea corporis inea corporis-vesicular inea corporis inea corporis ( aciei) Neonatal tinea inea incognito inea capitis Kerion inea capitis Id reaction to tinea inea corporis secondary to scalp in ection inea cruris inea pedis Onychomycosis White super cial onychomycosis inea manuum inea versicolor inea nigra inea imbricata Favus Congenital cutaneous candidiasis Candidiasis (moniliasis) Chronic mucocutaneous candidiasis

S e ctio n 7 De e p Fungal In e ctio ns Page 81 Figures 7-1 7-2, 7-3 7-4–7-7 7-8, 7-9

Chromoblastomycosis Coccidioidomycosis Sporotrichosis Mycetoma

S e ctio n 8 Bite s and In e s tatio ns Page 85 Figures 8-1–8-7 8-8 8-9 8-10, 8-11 8-12 8-13 8-14 8-15–8-25 8-26 8-27 8-28–8-31 8-32 8-33 8-34 8-35, 8-36 8-37 8-38 8-39 8-40

Insect “bites” Dermatitis caused by the common carpet beetle Spider bites Papular urticaria icks ick “bite” ick bite granuloma Scabies Postscabetic acropustulosis Scabies Pediculosis capitis (head lice) Pediculosis corporis Pediculosis pubis Macula ceruleae Myiasis Caterpillar dermatitis Dermatitis caused by blister beetles Seabather’s eruption Swimmer’s itch

x

Conte nts

8-41 8-42 8-43–8-46 8-47–8-48

T e sting o the Portuguese man-o -war T e e ect o contact with a sea urchin Larva migrans (creeping eruption) Onchocerciasis

S e ctio n 9 Ato pic De rm atitis Page 99 Figures 9-1–9-17 9-18, 9-19 9-20, 9-21 9-22 9-23 9-24, 9-25 9-26, 9-27

Atopic dermatitis Juvenile plantar dermatosis Frictional lichenoid dermatitis Atopic dermatitis with ollicular accentuation Lichen spinulosus Pityriasis alba Pompholyx (dyshidrotic eczema)

S e ctio n 10 Alle rg ic and Irritant Co ntact De rm atitis Page 107 Figure 10-1– 10-4 10-5 10-6 10-7 10-8 10-9 10-10 10-11–10-14 10-15, 10-16 10-17 10-18, 10-19 10-20 10-21, 10-22 10-23

Allergic contact dermatitis (Poison ivy) Allergic contact dermatitis (wet wipes) Allergic contact dermatitis (disposable diapers) Allergic contact dermatitis (mango) Allergic contact dermatitis (neomycin) Allergic contact dermatitis (shoes) Allergic contact dermatitis Nickel contact dermatitis Allergic contact dermatitis (Reaction to temporary tattoo) Irritant diaper dermatitis Erosive diaper dermatitis (dermatitis o Jacquet) Pseudoverrucous papules and nodules Lip licking dermatitis Shin guard dermatitis

S e ctio n 11 Pho to de rm ato s e s Page 115 Figure 11-1–11-4 11-5 11-6 11-7, 11-8 11-9, 11-10 11-11 11-12 11-13, 11-14 11-15, 11-16 11-17 11-18–11-20

Polymorphous light eruption Photoallergic dermatitis Photoxic dermatitis Erythropoietic protoporphyria NSAID-induced pseudoporphyria Actinic prurigo Actinic prurigo cheilitis Hydroa vaccini orme Juvenile spring eruption Photodermatitis (berloque dermatitis) Phytophotodermatitis

S e ctio n 12 Papulo s quam o us Dis e as e s Page 121 Figure 12-1–12-8 12-9 12-10–12-27 12-28–12-31 12-32–12-37 12-38–12-41 12-42–12-45 12-46–12-51 12-52–12-55 12-56–12-63 12-64–12-67 12-68, 12-69 12-70

Seborrheic dermatitis inea amiantacea Psoriasis Pustular psoriasis Pityriasis rubra pilaris Pityriasis rosea Pityriasis lichenoides chronica Pityriasis lichenoides et varioli ormis acuta (PLEVA, Mucha-Habermann disease) Lichen nitidus Lichen striatus Follicular mucinosis Porokeratosis Porokeratosis (porokeratosis o Mibelli)

xi

Conte nts

12-71 12-72, 12-73 12-74 12-75 12-76–12-85 12-86 12-87 12-88, 12-89

Linear porokeratosis Elastosis per orans serpiginosa Per orating olliculitis Reactive per orating collagenosis Lichen planus Actinic lichen planus Annular lichen planus Lichen planopilaris ( ollicular lichen planus)

S e ctio n 13 Nutritio nal, Me tabo lic, and Endo crine Dis e as e s Page 145 Figure 13-1–13-6 13-7–13-9 13-10 13-11, 13-12 13-13–13-15 13-16 13-17–13-21 13-22, 13-23 13-24 13-25–13-28

Acrodermatitis enteropathica Kwashiorkor Marasmus Pellagra Lipoid proteinosis Hurler syndrome Xanthomatosis Calcinosis cutis Progressive osseous heteroplasia Acanthosis nigricans

S e ctio n 14 Ge no de rm ato s e s Page 153 Figure 14-1–14-3 14-4 14-5–14-11 14-12–14-16 14-17–14-26 14-27 14-28, 14-29 14-30 14-31 14-32, 14-33 14-34, 14-35 14-36, 14-37 14-38–14-41 14-42–14-45 14-46–14-49 14-50–14-53 14-54–14-57 14-58, 14-59 14-60 14-61 14-62, 14-63 14-64–14-70 14-71 14-72–14-74 14-75–14-77

Pseudoxanthoma elasticum Cutis laxa Ehlers-Danlos syndrome Focal dermal hypoplasia (Goltz syndrome) Incontinentia pigmenti Wiskott-Aldrich syndrome Ataxia telangiectasia Bloom syndrome Rothmund-T omson syndrome (Poikiloderma congenitale) Cockayne syndrome Hypohidrotic ectodermal dysplasia Clouston syndrome Hay-Well syndrome (AEC) Pachyonychia congenita Dyskeratosis congenita Neuro bromatosis Neuro bromatosis (von Recklinghausen disease) Neuro bromatosis type I (von Recklinghausen disease) Neuro bromatosis type I Neuro bromatosis type I (Lisch nodule) Multiple endocrine neoplasia type 2 uberous sclerosis Buschke-Ollendor syndrome Basal cell nevus syndrome Xeroderma pigmentosum

S e ctio n 15 Ichthyo s e s and Dis o rde rs o Ke ratinizatio n Page 175 Figure 15-1–15-4 15-5 15-6–15-8 15-9–15-12 15-13, 15-14 15-15, 15-16 15-17–15-20 15-21–15-27

Ichthyosis vulgaris Harlequin-type ichthyosis Collodion baby Lamellar ichthyosis Lamellar icthyosis (cont’d.) Lamellar ichthyosis Recessive X-linked ichthyosis Epidermolytic ichthyosis ( ormerly epidermolytic hyperkeratosis)

x ii

Conte nts

15-28–15-30 15-31 15-32–15-34 15-35–15-40 15-41, 15-42 15-43, 15-44 15-45, 15-46 15-47–15-50 15-51, 15-52 15-53, 15-54 15-55–15-57 15-58 15-59, 15-60 15-61, 15-63 15-64

Nonbullous congenital ichthyosi orm erythroderma Erythrokeratoderma variabilis Progressive symmetric erythrokeratoderma Netherton syndrome Palmoplantar keratoderma Conradi-Hünermann syndrome Sjögren-Larsson syndrome Linear epidermal nevus Epidermal nevus syndrome Inf ammatory linear verrucous epidermal nevus (ILVEN) Darier disease (keratosis ollicularis) Acrokeratosis verruci ormis KID syndrome Keratosis pilaris Keratosis pilaris rubra aciei

S e ctio n 16 Urticarial, Purpuric, and Vas cular Re actio ns Page 193 Figure 16-1–16-4 16-5, 16-6 16-7–16-12 16-13–16-20 16-21–16-23 16-24 16-25, 16-26 16-27, 16-28 16-29–16-32 16-33, 16-34 16-35, 16-36 16-37–16-40 16-41, 16-42 16-43, 16-44 16-45–16-47 16-48, 16-49

Urticaria Physical urticarias Erythema multi orme Stevens-Johnson syndrome/toxic epidermal necrolysis Urticaria multi orme Sweet syndrome (acute ebrile neutrophilic dermatosis) Erythema annulare centri ugum Erythema elevatum diutinum Progressive pigmented purpura (Schamberg disease) Acute hemorrhagic edema o in ancy raumatic purpura Henoch-Schönlein purpura Purpura ulminans Aphthous stomatitis Behçet syndrome Erythema ab igne

S e ctio n 17 Bullo us , Pus tular, and Ulce rating Dis e as e s Page 207 Figure 17-1–17-4 17-5 17-6 17-7, 17-8 17-9–17-12 17-13, 17-14 17-15 17-16–17-18 17-19 17-20–17-22 17-23–17-25 17-26 17-27–17-29 17-30 17-31–17-33 17-34–17-37

Pemphigus vulgaris Pemphigus vegetans Familial benign chronic pemphigus (Hailey-Hailey disease) Subcorneal pustulosis (Sneddon-Wilkinson disease) Pemphigus oliaceus Bullous pemphigoid Vulvar pemphigoid Epidermolysis bullosa simplex (EBS) Epidermolysis bullosa simplex (Dowling-Meara) Epidermolysis bullosa, junctional type (JEB) Epidermolysis bullosa recessive dystrophic type (RDEB) Epidermolysis bullosa dominant dystrophic type, generalized (DDEB) Dystrophic epidermolysis bullosa, dominant type (DDEB) Epidermolysis bullosa with congenital absence o skin Dermatitis herpeti ormis Linear Ig A dermatosis (chronic bullous dermatosis o childhood)

S e ctio n 18 Cutane o us Mani e s tatio ns o HIV Dis e as e Page 219 Figure 18-1 18-2 18-3

Cutaneous mani estations o HIV in ection (chronic varicella zoster in ection) Cutaneous mani estations o HIV in ection (herpes zoster in ection) Cutaneous mani estations o HIV in ection (scarring rom herpes zoster)

x iii

Conte nts

18-4 18-5 18-6 18-7 18-8 18-9 18-10 18-11, 18-12

Cutaneous mani estations o Cutaneous mani estations o Cutaneous mani estations o Cutaneous mani estations o Cutaneous mani estations o Cutaneous mani estations o Cutaneous mani estations o Cutaneous mani estations o

HIV in HIV in HIV in HIV in HIV in HIV in HIV in HIV in

ection (candidal paronychias and nail dystrophy) ection (drug eruption) ection (molluscum contagiosum) ection (chronic herpetic gingivostomatitis) ection (seborrheic dermatitis) ection (condylomata acuminata) ection (widespread f at warts) ection (psoriasis)

S e ctio n 19 Cutane o us Mani e s tatio ns o Sys te m ic Dis e as e Page 223 Figure 19-1–19-6 19-7 19-8–19-11 19-12–19-15 19-16–19-25 19-26, 19-27 19-28 19-29 19-30, 19-31 19-32, 19-33 19-34, 19-35 19-36, 19-37 19-38, 19-39 19-40–19-45 19-46, 19-47

Systemic lupus erythematosus Systemic lupus erythematosus vasculitis Neonatal lupus erythematosus Discoid lupus erythematosus Dermatomyositis Scleroderma (progressive systemic sclerosis) Cutaneous expression o rheumatic ever Cutaneous expression o rheumatoid arthritis Cutaneous expressions o polyarteritis nodosa Sarcoidosis Pernio Cutaneous mani estations o Crohn disease Pyoderma gangrenosum Kawasaki disease Necrobiosis lipoidica

S e ctio n 20 Dis o rde rs o the De rm is (Inf ltrate s , Atro phie s , and No dule s ) Page 237 Figure 20-1, 20-2 20-3–20-5 20-6 20-7, 20-8 20-9, 20-10 20-11 20-12–20-15 20-16 20-17, 20-18 20-19–20-21 20-22, 20-23 20-24–20-26 20-27, 20-28 20-29 20-30–20-37 20-38, 20-39 20-40, 20-41 20-42, 20-43 20-44–20-46 20-47 20-48–20-51 20-52, 20-53 20-54, 20-55 20-56–20-59 20-60, 20-61 20-62 20-63 20-64, 20-65 20-66–20-68

Morphea Morphea (linear) Morphea (linear, en coup de sabre) Morphea Generalized morphea Atrophoderma (Pasini-Pierini) Lichen sclerosus et atrophicus Lichen sclerosus et atrophicus (Balanitis xerotica obliterans) Anetoderma (macular atrophy) Striae distensae Connective tissue nevus Granuloma annulare Subcutaneous granuloma annulare Eruptive granuloma annulare Juvenile xanthogranuloma Dermato broma Granular cell tumor Benign cephalic histiocytosis Keloids Fibrous hamartoma o in ancy Digital brous tumor o childhood In antile myo bromatosis Mastocytoma Mastocytosis Bullous mastocytosis (Di use cutaneous mastocytosis) Nevus lipomatosus super cialis Leiomyoma Smooth muscle and pilar hamartoma Lymphomatoid papulosis

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Conte nts

S e ctio n 21 Drug Eruptio ns Page 257 Figure 21-1–21-3 21-4 21-5–21-8 21-9 21-10 21-11, 21-12 21-13, 21-14

Drug eruptions Urticaria multi orme Fixed drug eruption Hypersensitivity syndrome Drug-induced gingival overgrowth Iododerma and bromoderma Acute generalized exanthematous pustulosis (AGEP)

S e ctio n 22 Panniculo pathie s Page 263 Figure 22-1, 22-2 22-3 22-4 22-5, 22-6 22-7, 22-8 22-9 22-10 22-11 22-12

Subcutaneous at necrosis o the newborn Subcutaneous at necrosis Sclerema neonatorum Erythema nodosum Panniculitis rom cold Lipoatrophy (localized) Lipoatrophy secondary to reticular hemangioma Progressive partial lipodystrophy Acquired generalized lipodystrophy

S e ctio n 23 He m ang io m as and Vas cular and Lym phatic Dis o rde rs Page 267 Figure 23-1 23-2, 23-3 23-4 23-5 23-6–23-8 23-9, 23-10 23-11–23-20 23-21–23-24 23-25, 23-26 23-27 23-28 23-29, 23-30 23-31, 23-32 23-33, 23-34 23-35, 23-36 23-37 23-38 23-39 23-40 23-41, 23-42 23-43, 23-44 23-45 23-46–23-48 23-49, 23-50 23-51, 23-52 23-53, 23-54 23-55

Cutis marmorata Cutis marmorata telangiectatica congenita Livedo reticularis Nevus simplex (salmon patch) Port-wine stain Sturge-Weber syndrome Hemangioma Hemangioma (ulcerated) Rapidly involuting congenital hemangioma (RICH) Noninvoluting congenital hemangiomas (NICH) Disseminated hemangiomatosis PHACES syndrome Pyogenic granuloma Glomovenous mal ormation (glomus tumor) Angiokeratoma (angiokeratoma o Mibelli) Solitary angiokeratoma Angiokeratoma circumscriptum Fabry disease (angiokeratoma corporis di usum) Spider angioma (nevus araneus) Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) Klippel- rénaunay-Weber syndrome Milroy disease Lymphangioma Vascular mal ormations u ed angioma Kaposi orm hemangioendothelioma Unilateral nevoid telangiectasia

S e ctio n 24 Ne o plas tic Dis o rde rs Page 283 Figure 24-1, 24-2 24-3 24-4 24-5, 24-6 24-7–24-14 24-15, 24-16 24-17, 24-18 24-19, 24-20

Leukemia cutis Hodgkin disease Chloroma Neuroblastoma, metastatic Langerhans cell disease Congenital sel -healing reticulohistiocytosis (Hashimoto-Pritzker disease) Basal cell carcinoma Gra -versus-host disease (GVHD)

xv

Conte nts

24-21–24-23 Hydroa vaccini orme-like cutaneous -cell lymphoma (C CL) 24-24 Rosai-Dor man disease (sinus histiocytosis with massive lymphadenopathy) 24-25 Dermato brosarcoma protuberans

S e ctio n 25 Adne xal Dys plas ias Page 291 Figure 25-1–25-7 25-8 25-9, 25-10 25-11, 25-12 25-13, 25-14 25-15 25-16, 25-17 25-18, 25-19 25-20, 25-21 25-22, 25-23 25-24 25-25 25-26, 25-27 25-28, 25-29

Nevus sebaceous Clear cell hidradenoma Syringoma Dermoid cyst Epidermal cyst Congenital giant milium o the anterior neck Pilomatricoma (benign calci ying epithelioma o Malherbe) Pilomatricoma richoepithelioma Eruptive vellus hair cysts Steatocystoma multiplex Eccrine poroma Palmoplantar eccrine hidradenitis Eccrine angiomatous hamartoma

S e ctio n 26 Be nig n and Malig nant Pig m e nte d Le s io ns Page 301 Figure 26-1 26-2 26-3, 26-4 26-5 26-6 26-7, 26-8 26-9, 26-10 26-11, 26-12 26-13 26-14 26-15, 26-16 26-17, 26-18 26-19, 26-20 26-21 26-22 26-23–26-26 26-27, 26-28 26-29, 26-30 26-31 26-32 26-33 26-34, 26-35

Ephelis Lentigo Peutz-Jeghers syndrome Multiple lentigines syndrome Junctional nevus Compound nevus Intradermal nevus Melanonychia Eclipse scalp nevus Cockarde nevus Speckled lentiginous nevus (Nevus spilus) Halo nevus Spitz nevus Multiple and agminated Spitz nevi Pigmented spindle cell nevus Congenital melanocytic nevus Malignant melanoma Dermal melanocytosis (mongolian spot) Nevus o Ota Nevus o Ito Blue nevus Becker nevus

S e ctio n 27 Mis ce llane o us Pig m e ntary Dis o rde rs Page 311 Figure 27-1– 27-3 27-4 27-5 27-6 27-7, 27-8 27-9–27-11 27-12–27-17 27-18 27-19 27-20, 27-21 27-22 27-23, 27-24

Pigmentary mosaicism Nevus depigmentosus (achromicus) Nevus anemicus Carotenemia Waardenburg syndrome Piebaldism Vitiligo Albinism Chédiak-Higashi syndrome Postinf ammatory hypopigmentation Progressive macular hypomelanosis Phakomatosis pigmentovascularis

xvi

Conte nts

S e ctio n 28 De rm atitis Arte acta Page 319 Figure 28-1–28-4 28-5–28-11 28-12 28-13 28-14 28-15 28-16

Child abuse Factitial dermatitis Cupping Senna laxative-induced blistering dermatitis Pseudoainhum alon noir (black heel) attoos

S e ctio n 29 Dis o rde rs o Nails and Hair Page 325 Figure 29-1 29-2 29-3 29-4 29-5 29-6 29-7, 29-8 29-9 29-10 29-11 29-12 29-13–29-16 29-17 29-18 29-19, 29-20 29-21 29-22–29-27 29-28 29-29 29-30 29-31 29-32, 29-33 29-34 29-35 29-36, 29-37 29-38, 29-39 29-40, 29-41 29-42, 29-43 29-44 29-45

Clubbed nails rachyonychia raumatic onychodystrophy (Habit tic de ormity) Dystrophia unguis mediana canali ormis Leukonychia totalis Leukonychia striata Onycholysis Onychomadesis Onychoschizia Beau lines Discoloration o nail plates Nail-patella syndrome Congenital ingrown toenail Ingrown toenail richotillomania raumatic alopecia Alopecia areata Alopecia universalis Alopecia areata (recovered) emporal triangular alopecia Uncombable hair syndrome Monilethrix richorrhexis nodosa Monilethrix and trichorrhexis nodosa (magni ed appearance o hair sha s) Pili torti Loose anagen syndrome richothiodystrophy Nevoid hypertrichosis Wooly hair nevus Cutis verticis gyrata

S e ctio n 30 Mis ce llane o us Ano m alie s Page 339 Figure 30-1–30-8 30-9, 30-10 30-11 30-12 30-13 30-14 30-15–30-17 30-18 30-19, 30-20 30-21 30-22 30-23, 30-24 30-25 30-26 30-27, 30-28 30-29

Aplasia cutis congenita Fetus papyraceus Aplasia cutis congenita limited to legs and eet Supernumerary digits Amputation neuroma Supernumerary nipple Auricular tags Dental sinus Branchial-cle cysts T yroglossal cyst Anomalies o umbilical maldevelopment Omphalomesenteric remnants Umbilical granuloma Sucking blister Geographic tongue Fordyce condition

x v ii

Conte nts

30-30 30-31 30-32 30-33 30-34 30-35, 30-36 30-37, 30-38 30-39, 30-40 30-41 30-42 30-43, 30-44 30-45 30-46 30-47, 30-48 30-49, 30-50 30-51, 30-52

Inde x Page 353

yson glands Hyperhidrosis Aquagenic wrinkling o the palms Mucocele Anterior cervical hypertrichosis Median raphe cyst o the scrotum Calci ed heel stick nodule Calci ed ear nodule In antile pyramidal perianal protrusion Knuckle pads Precalcaneal brolipomatous hamartoma erra rma- orm dermatosis Writing callus Subungual exostosis Conf uent and reticulated papillomatosis Nasal crease papules

PREFACE

T e rst edition o the Color Atlas of Pediatric Dermatology was published in 1975, during the very in ancy o our specialty. T e book was the product o three brilliant physicians, Drs. Samuel Weinberg, Morris Leider, and Lewis Shapiro, and each brought a unique talent to its creation. Dr. Shapiro was a dermatopathologist, dermatologist, and highly regarded teacher at Columbia University. For the early editions, he contributed beauti ul photomicrographs to accompany the clinical pictures. His contributions to the book in its earliest stages, and to the dermatopathology literature as a whole, are o great value. Dr. Leider was an illustrious and longtime member o the Department o Dermatology at New York University. He was a wonder ul amily riend during my early childhood, and he gave me the rst edition o the book as a medical school graduation gi in 1975 (NSP). Dr. Leider was the author o a dermatologic dictionary and prided himsel on the literary use o words in the medical context. His unique and f owery writing style can still be ound in various nooks and crannies o the book, and a particularly wonder ul example is located beneath Fig. 16-36. Dr. Weinberg was one o the ounding members o the Society or Pediatric Dermatology and was or many years the Chie o Pediatric Dermatology at Bellevue Hospital in New York. T e Color Atlas of Pediatric Dermatology was o enormous importance

x ix

to him throughout his whole li e, and he guided its content in a remarkably knowledgeable and thoughtul ashion. He was a wonder ul teacher, mentor, and riend to both o us or many years and we sorely missed his insight and humor during the preparation o this edition. In Dr. Leider’s oreword to the rst edition o the atlas, we are given a wonder ul insight into the special and, we imagine, laughter- lled relationship o these physicians and into their method o conf ict resolution. Dr. Leider wrote, “We will spare the reader the gory details o our violent arguments by saying that all contended matter was settled by a ‘majority o one’ by Dr. Shapiro when it was purely histologic, by Dr. Leider when it was purely literary, and by Dr. Weinberg when it was purely pediatric. For the rest, a true majority ruled.” We are delighted that the Color Atlas of Pediatric Dermatology has been in print or over 40 years and are proud to be part o this long tradition. Once again, we ask the reader to bear in mind that this volume is not a textbook and that it should be used in conjunction with one o the several comprehensive re erences in pediatric dermatology. It is our hope that this atlas will be o practical use to all health practitioners who are involved in the care o children. Neil S. Prose Leonard Kristal

SECTION

1 Benign Neonatal Dermatoses

2

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 1-1

Fig ure 1-2

Erythema toxicum neonatorum T is very common and completely benign condition usually arises in the rst 2 days o li e. It is seen in about 30% to 50% o healthy newborns and occurs less requently in preterm in ants. Rarely the onset occurs up to 14 days o age.

T e lesions are erythematous macules, within which papules (Fig. 1-1) and pustules (Fig. 1-2) may develop. T e trunk is the most common site, but all other body sur aces, except or the palms and soles, may be involved. In rare cases, these lesions may occur in plaques.

Fig ure 1-3

Fig ure 1-4

Erythema toxicum neonatorum Occasionally, this unimportant eruption must be dif erentiated rom more serious in ectious processes, such as neonatal herpes simplex. zanck smear o a pustule o erythema toxicum neonatorum will reveal numerous eosinophils but no multinucleated giant cells or bacteria.

In some newborns, peripheral eosinophilia is also present. T e cause o this condition is not known, and it resolves spontaneously within 10 days. No treatment is required.

3

Se ction 1 . Be nign Ne onatal De rm atos e s

Fig ure 1-5

Fig ure 1-6

Transient neonatal pustular melanosis T is is a benign neonatal dermatosis that is most common among children with more dark-colored skin. T e original lesion is a vesiculopustule, which may be present at birth. T is small blister quickly ruptures and leaves a typical collarette o super cial scale. Both intact pustules and collarettes are seen in the newborn in Figs. 1-5 and 1-6.

Figures 1-6 and 1-7 show the brownish-pigmented macules that may develop at the site o resolving lesions. T ese macules may be sparse or numerous and resolve without residua over a period o several weeks to several months.

Fig ure 1-7

Fig ure 1-8

Transient neonatal pustular melanosis In some in ants, the pustule and collarette stages seem to occur in utero, and the sole cutaneous mani estations are the typical macules (Fig. 1-8). Lesions o transient neonatal pustular melanosis avor the orehead, neck, chin, and lower back but may be very widespread and may involve the palms and soles.

Scraping the base o an unroo ed pustule reveals polymorphonuclear leukocytes but no bacteria, pseudohyphae, or multinucleated giant cells. A biopsy o a pustule, which is rarely necessary, shows an intraepidermal collection o polymorphonuclear leukocytes.

SECTION

2 Milia, Miliaria, and Pustular and Acnei orm Disorders

6

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 2-1

Fig ure 2-2

Milia A milium is a white papule, 1 to 2 mm in size, composed o laminated, keratinous material and situated as a solid cyst in a pilosebaceous ollicle. Milia are airly common on the brow, glabella, and nose in newborn in ants and in such in ants tend to disappear quickly and spontaneously. T ere may be ew or many, and they may develop later in in ancy, in childhood, and in adolescence. In older children and adolescents, they tend to persist, may precede acne or be associated with incipient acne

and commonly develop on or around the eyelids. Milia may be ablated, i desirable, by delicate incision and expression o the keratinous content. Lesions that are treated do not recur, but i new lesions appear, they have to be treated in the same way. T e operation is trivial and uncomplicated. T ere are no preventive measures.

Fig ure 2-3

Eosinophilic pustular olliculitis o in ancy Children with this rare disorder develop repeated crops o pruritic erythematous papules, yellow or white pustules, which vary in size rom 1 to 3 mm. Most lesions are located on the scalp and distal extremities. zanck smear may reveal numerous eosinophils, and there may also be a peripheral eosinophilia when aring. Eosinophilic pustular olliculitis is associated with no systemic symptoms and eventually resolves spontaneously. T erapy with topical steroids is bene cial.

Se ction 2 . Milia, Miliaria, and Pus tular and Acne iform Dis orde rs

7

Fig ure 2-4

Fig ure 2-5

In antile acropustulosis T is cutaneous disorder is characterized by recurrent episodes o intensely pruritic pustules and papulovesicles on the hands and eet. Lesions are most common on the palms and soles but may be seen on the dorsal sur aces as well.

Lesions may also occur on the ankles, orearms, and, rarely, the ace, scalp, and upper trunk. T e age at onset is typically between 2 and 10 months. Individual episodes last or 7 to 10 days and may recur as o en as every 2 weeks at the beginning o the disease. Episodes tend to become less requent and severe over time.

Fig ure 2-6

Fig ure 2-7

In antile acropustulosis Stained smears o an individual lesion will reveal numerous neutrophils, although eosinophils may be present early in the course o the disorder. In antile acropustulosis may also be seen a er scabies in estation in in ants (“postscabies syndrome”).

Figure 2-7 shows involvement o the orehead in a patient with in antile acropustulosis. T e disease resolves spontaneously by 2 to 3 years o age. T e individual lesions in this condition may resolve with scale and postin ammatory hyperpigmentation.

8

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 2-8

Fig ure 2-9

Miliaria crystallina T e lesions in this condition are small, clear, thin-roo ed vesicles that develop when the sweat duct is obstructed within the stratum corneum. T ey occur a er sunburn or in response to excessive sweating in high environmental heat and humidity. Fever may also be a cause.

T e scalp, ace, trunk, and intertriginous areas are sites o lesions. Itching is not a symptom. T e vesicles resolve rapidly with the elimination o the causative environmental actor.

Fig ure 2-10

Miliaria rubra (prickly heat) T is is the most common orm o miliaria. It occurs when there is plugging o the eccrine ducts and release o sweat into the adjacent skin. Miliaria rubra is characterized by discrete erythematous papules and papulovesicles. T e orehead, upper trunk, and intertriginous areas are commonly a ected. Unlike miliaria crystallina, miliaria rubra is characterized by spasmodic pricking sensations. A decrease in environmental heat and humidity is the only treatment required.

Se ction 2 . Milia, Miliaria, and Pus tular and Acne iform Dis orde rs

9

Fig ure 2-11

Fig ure 2-12

Fox-Fordyce disease (apocrine miliaria) T is chronic and intensely pruritic papular eruption is localized to the axillae, areolae, and pubic areas where apocrine glands are ound. It occurs almost exclusively in young women, requently with the onset during adolescence. T e ollicular papules result rom the obstruction o the intraepidermal sweat duct, with the release o apocrine sweat into the surrounding skin.

Figure 2-11 shows the process in an axilla; Fig. 2-12 shows it in the pubic area. T e etiology o Fox-Fordyce disease is unknown and the treatment is dif cult. opical retinoids, hormonal therapy, and antimicrobial therapy are sometimes help ul. Pimecrolimus, a topical immunomodulator, has recently been shown to be bene cial.

Fig ure 2-13

Fig ure 2-14

Neonatal cephalic pustulosis T is disorder is characterized by the development o numerous very small erythematous papulopustules over the scalp, ace, and neck. Lesions usually develop during the second or third week o li e. Researchers believe that this eruption is identical to that which was previously termed

as neonatal acne. Recent research suggests that the cause is the lipophilic yeast, Malassezia. M ur ur or M sympodialis, can be isolated rom the skin o most patients. opical ketoconazole is a sa e and e ective treatment.

10

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 2-15

Fig ure 2-16

Neonatal and in antile acne Mild comedonal acne is airly common in the newborn. T e typical eruption consists o closed comedones. Open comedones, in ammatory papules and pustules, and small cysts may also occur. Neonatal acne is due to the stimulation o sebaceous glands by androgens rom both mother and in ant.

T e lesions o neonatal acne usually resolve during the rst ew months o li e. Acne, in varying degrees o severity, may also appear in in ants a er the neonatal period. T is orm o in antile acne may persist or 1 or 2 years and may rarely eventuate in scarring.

Fig ure 2-17

Fig ure 2-18

Neonatal and in antile acne Children with an early onset o acne and a strong amily history are particularly at risk or a severe course o the disease during puberty. Most cases o neonatal and in antile acne do not require treatment. I necessary, a mild benzoyl peroxide preparation may be used.

In antile cystic acne Comedones generally predominate in in antile acne, although more in ammatory papules and pustules may be seen. Rarely, an in ant may develop cystic nodules, as seen in Fig. 2-18, that occasionally heal with scarring. In ants with severe acne should be evaluated or sexual precocity or abnormal virilization.

Se ction 2 . Milia, Miliaria, and Pus tular and Acne iform Dis orde rs

11

Fig ure 2-19

Fig ure 2-20

Acne vulgaris T e common varieties o acne generally begin to develop in late childhood or early adolescence. Acne during adolescence is caused by the e ect o androgenic hormones on the pilosebaceous unit. T e increased activity o the sebaceous gland provides a substrate or Propionibacterium acnes, whose

lipolytic enzymes convert triglycerides in sebum to ree atty acids. Abnormal keratinization in the pilosebaceous ollicle also plays a role in the development o acne. T e earliest lesions are open or closed comedones. Figures 2-19 and 2-20 show the comedo stage, with open and closed comedones represented.

Fig ure 2-21

Fig ure 2-22

Acne vulgaris Comedones may be ound in the ears, most commonly in the conchal area as seen in Fig. 2-21. Comedones in this area can be quite large and can at times resolve with pitted scarring.

Figure 2-22 shows open and closed comedones, a ew in ammatory papules and pitted scarring.

12

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 2-23

Fig ure 2-24

Acne vulgaris Many cases o acne progress rom open and closed comedones (blackheads and whiteheads) to in ammatory orms that are marked by papules, pustules, and cysts. Figure 2-23 shows the beginning o progression to in ammatory papules and pustules. Figure 2-24 shows the progression rom the comedonal to a more severe in ammatory phase o acne.

opical therapies are aimed at decreasing skin colonization by P acnes (topical antibiotics) and at normalizing keratinization within the ollicle (tretinoin). As acne becomes more in ammatory, systemic antibiotics may be needed to decrease the in ammation and to prevent possible scarring. Avoidance o irritating soaps and scrubbing is advised.

Fig ure 2-25

Fig ure 2-26

Cystic acne Figure 2-25 shows a combination o in ammatory acne with cysts. Figure 2-26 indicates the urther progression with numerous in ammatory cystic nodules. Severe cystic acne is more dif cult to control or cure and may cause scarring. Although, topical therapy with combinations o antibiotics and topical retinoids is o value, systemic antibiotics, such as doxycycline and tetracycline may also be required because o their antibacterial and anti-in ammatory properties.

T e intralesional injection o corticosteroids may also be help ul. Isotretinoin is indicated or nodulocystic acne that may cause scarring or recalcitrant acne that is not responding to therapy. Side e ects o this therapy range rom dry lips and skin to elevation o cholesterol and triglycerides. In addition, all emale patients must be advised that isotretinoin is a potent teratogen and that pregnancy must be avoided.

Se ction 2 . Milia, Miliaria, and Pus tular and Acne iform Dis orde rs

13

Fig ure 2-27

Acne conglobata In conglobate acne, the most severe orm o acne, cysts tend to be large, irregular, and intercommunicating and tend to result in severe scarring. Severe cystic acne can sometimes be controlled with topical therapy and systemic antibiotics. T e patient who does not respond to these measures may be a candidate or isotretinoin.

Fig ure 2-28

Fig ure 2-29

Scarring ollowing acne Atrophic scars, hypertrophic scars, and keloids ollowing resolution o in ammatory lesions o acne are an uncommon but particularly distressing complication. T e chest and upper back are the sites o predilection. Figure 2-28 shows atrophic scarring with ongoing in ammatory lesions. Figure 2-29 shows the keloids that may result. Keloids occur

less commonly on the ace. T e tendency toward keloid ormation is more common in A rican American adolescents and is sometimes amilial. Success ul control o the acne itsel , through topical or systemic therapy, may minimize the extent o uture scarring. T e use o intralesional corticosteroids is the most e ective treatment o keloids once they have ormed.

14

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 2-30

Fig ure 2-31

Acne and precocious puberty rom a pinealoma In this 4-yearold child, persistent acne, precocious puberty, and requent headache were the presenting signs and symptoms o a pinealoma. Occasionally, acne in a preadolescent may be an indication o an endocrine abnormality in which either androgen or glucocorticoids are present in excess. For example, a typical eruption o monomorphic ollicular papules, usually concentrated on the back and chest, is seen in Cushing disease.

Steroid acne (dexamethasone) T is orm o acne may be caused by systemic or topical corticosteroids, as is seen in Fig. 2-31 o a patient receiving systemic dexamethasone. T e eruption is monomorphous, characterized by the presence o small erythematous papules or pustules primarily seen on the upper trunk, arms, neck, and, less commonly, the ace.

Fig ure 2-32

Fig ure 2-33

Figure 2-32 shows numerous monomorphous ollicular papules in a patient undergoing systemic steroid therapy with dexamethasone.

Steroid acne (dexamethasone) Figure 2-33 shows a young girl who has applied a uorinated topical steroid cream to her ace. T is has resulted in a monomorphous eruption o in ammatory acnei orm papules.

Se ction 2 . Milia, Miliaria, and Pus tular and Acne iform Dis orde rs

15

Fig ure 2-34

Fig ure 2-35

Hidradenitis suppurativa T is condition is a chronic, recurrent in ammatory process o unknown etiology that involves the ollicular epithelium o apocrine bearing areas. T e avored locations are the axillae, groin, and buttocks. T e disease begins just be ore or during puberty and persists, with remissions and exacerbations, or years. It is more common in women and is

sometimes associated with acne conglobata and dissecting cellulitis o the scalp. Worsening o the disease may be seen during summer months or at the time o menstruation. Hidradenitis suppurativa is aggravated by obesity. T e involved areas in the axilla or groin may present with pustules, nodules, abscesses, and sinus tracts.

Fig ure 2-36

Fig ure 2-37

Hidradenitis suppurativa In Fig. 2-36, the condition has partially, and temporarily, abated. Corded hypertrophic and keloidal scarring has developed. T e treatment o hidradenitis suppurativa is o en dif cult. opical and systemic antibiotics are most commonly used; a combination o oral ri ampin and clindamycin has been documented to be e ective. reatment with biologic agents may also provide relie in patients with severe involvement.

Rosacea T is is an in ammatory condition o the mid ace characterized by the presence o erythema, papules, pustules, telangiectasias, and, in the later stages, hyperplasia o the sebaceous glands o the nose. T e absence o comedones helps distinguish this condition rom acne vulgaris, although the two conditions may coexist. Although usually seen in middle age, this condition may start in late adolescence.

16

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 2-38

Fig ure 2-39

Periorif cial dermatitis T is condition is a chronic eruption o ne papules and pustules located on the skin around the mouth and nose and sometimes around the eyes. T e condition may initially present with perinasal scaling, which then progresses to involve the perioral area.

T e etiology o this condition is not completely understood. It usually begins in early childhood, with men and women equally a ected. It may be more common in children with skin o color.

Fig ure 2-40

Fig ure 2-41

Periorif cial granulomatous dermatitis opical steroids may cause severe worsening o this disorder. In most instances, perioral granulomatous dermatitis responds to oral erythromycin or to the application o topical metronidazole.

In most cases, this is a clinical diagnosis and biopsy need not be per ormed. However, the lesions do have a granulomatous in ltrate, and, on the basis o both the appearance and the histology, the condition may be con used with sarcoid.

Se ction 2 . Milia, Miliaria, and Pus tular and Acne iform Dis orde rs

17

Fig ure 2-42

Periorif cial dermatitis Occasionally an in ant or young child may present with an in ammatory papular or papulopustular eruption in the in raorbital area. T is may be the initial presentation o periori cial dermatitis. In a brie period o time, most patients develop a more typical eruption located around the mouth.

Fig ure 2-43

Fig ure 2-44

Dissecting cellulitis o the scalp T is condition was originally called olliculitis et peri olliculitis capitis abscedens et suf odiens, which translates into an in ammation in and around hair ollicles o the scalp that ows (pus) and channels under or through (tissue). T is chronic condition resembles, and sometimes accompanies, acne conglobata and hidradenitis suppurativa (the ollicular occlusion triad). Like them, it is marked by in ammation, purulence, intercommunicating abscesses, cysts, sinuses,

and scarring. T is disease appears to be somewhat more common among A rican Americans, and the onset may occur during adolescence. A number o therapies are routinely used. T ese include topical and systemic antibiotics, incision and drainage o abscesses, and intralesional steroids. More recently, there is some evidence that treatment with biologic agents may be o bene t. T ere are usually many remissions and exacerbations, and the process o en eventuates in a scarring alopecia.

SECTION

31 Bacterial In ections

20

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 3-1

Fig ure 3-2

Impetigo Impetigo is a primary super cial in ection o the skin. It is more prevalent in humid climates and occurs most commonly in the summer months. rauma to the skin, such as a small abrasion or insect bite, sometimes provides the site o entry or the in ective bacteria. T e lesions evolve rom discrete small vesicles into pustules. T e uid content o the primary lesions dries into a thick yellowish crust (Fig. 3-1), and removal o the crust may reveal bright-red and shiny erosions (Fig. 3-2).

T e most common cause o impetigo is Staphylococcus aureus. Because the “honey-crusted” lesions o impetigo may be caused by a combination o S aureus and Streptococcus pyogenes, systemic antibiotic therapy should be e ective against both organisms. T e use o topical mupirocin ointment appears to be an e ective treatment and may replace the need or systemic therapy in some patients with localized lesions.

Fig ure 3-3

Fig ure 3-4

Bullous impetigo T is orm o impetigo consists o accid blisters that quickly rupture and evolve into super cial round or oval erosions with a varnished sur ace and minimal crust. Blisters are caused by the local e ect o staphylococcal toxin. Figure 3-3 shows blisters and super cial erosions.

Figure 3-4 shows the collarettes o scale ollowing rupture o the bullae. Bullous impetigo is associated with a pure culture o S aureus. Oral treatment with dicloxacillin or a cephalosporin is an e ective mode o therapy. I methicillin-resistant Staphylococcus aureus (MRSA) is suspected, oral clindamycin is requently recommended, and can be used pending results o culture.

21

Se ction 3 . Bacte rial Infe ctions

Fig ure 3-5

Fig ure 3-6

Impetiginization T is is the term or impetigo imposed upon preexisting dermatoses, most commonly insect bites and atopic dermatitis. Eruptions that are pruritic are particularly susceptible to secondary in ection. T e most common organisms are S pyogenes and S aureus. Figure 3-5 shows a case o impetiginized atopic dermatitis. T e development o such “honey-crusted” lesions in a child with eczema suggests the need or systemic antibiotic therapy.

Ecthyma Ecthyma occurs when there is ulceration beneath the sur ace o a skin in ection. I impetigo is in ection by streptococci and/or staphylococci super cially in the epidermis, ecthyma is in ection by the same organisms through the entire thickness o the epidermis (0.1 mm) to the upper reaches o the dermis (perhaps to a depth o 0.5 mm). Clinically, there is o en a rm crust covering a super cial ulcer, surrounded by erythema.

Fig ure 3-7

Fig ure 3-8

Staphylococcal scalded skin syndrome T is eruption occurs most commonly in children under the age o 5 years. It is characterized by a generalized tender, macular erythema, which is most prominent on the skin around the mouth and nose and in intertriginous areas. Within 1 or 2 days, the rash begins to peel. ypically, the large super cial accid bullae (“scalded skin”) are quickly unroo ed, revealing areas o slightly erythematous and shiny skin. T ese areas crust and then heal. Children with this syndrome are o en extremely irritable and ebrile, but the

overall prognosis is good. Figures 3-7 and 3-8 illustrate supercial blistering and erythema around the mouth. T e scalded skin syndrome is caused by an epidermolytic toxin that may be produced by several strains o S aureus. T ese causative organisms may be present in the nose, throat, conjunctiva, or an in ected wound. Staphylococcal scalded skin syndrome resolves without scarring within a period o 2 weeks. reatment consists o appropriate supportive care and penicillinase-resistant antibiotics.

22

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 3-9

Fig ure 3-10

Staphylococcal scalded skin syndrome Figure 3-9 shows a generalized light-colored erythema which is accentuated in skin olds. T e staphylococcal toxin ex oliatin may sometimes produce extensive areas o desquamation. Other clues to diagnosis include areas o denuded skin in sites o anatomic stress, and skin tenderness.

reatment in severe cases consists o intravenous antibiotics that are e ective against strains o S aureus that are prevalent in the geographic area. In ants with this degree o involvement (Fig. 3-10) must be managed care ully with respect to uid and electrolyte levels.

Fig ure 3-11

Fig ure 3-12

Staphylococcal scalded skin syndrome Scalded skin syndrome must be di erentiated rom scarlet ever, Kawasaki disease, toxic shock syndrome, and drug-induced toxic epidermal necrolysis.

Chancri orm pyoderma In ection with a Staphylococcus, or more o en with organisms such as Pseudomonas aeruginosa or Proteus and combinations thereo , can result in chancri orm ulcers. T ese lesions are more dif cult to treat. In addition to e ective systemic antibiotics, attention must be paid to skin care o the entire diaper area, and topical antibiotics may be required.

23

Se ction 3 . Bacte rial Infe ctions

Fig ure 3-13

Fig ure 3-14

Folliculitis T is is a common orm o bacterial skin in ection in both children and adolescents. ypically, the lesions are erythematous papules or pustules, arising at the openings o hair ollicles. Pruritus or mild discom ort may be associated with the in ection.

Involvement o the buttocks and perineum is particularly common in in ants and young children (Fig. 3-14). In ants may be predisposed to olliculitis in this area secondary to occlusion by diapers.

Fig ure 3-15

Fig ure 3-16

Folliculitis, shown in Fig. 3-15 in the beard area, is most commonly caused by in ection with S aureus and responds to treatment with oral antibiotics that cover this organism. In areas where methicillin-resistant organisms are common, antibiotic therapy needs to be adjusted accordingly.

Hot tub olliculitis T is condition is seen a er immersion in a hot tub in which gram-negative organisms, predominantly Pseudomonas species, proli erate as a result o improper maintenance. Patients develop numerous discrete erythematous papules and pustules on the upper trunk, groin, buttocks, and thighs. Lesions may be tender. T e eruption is sel -limited, although topical gentamicin and/or diluted white vinegar soaks may hasten resolution. Hot tubs must be properly cleaned and maintained.

24

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 3-17

Fig ure 3-18

Furuncle A uruncle is a skin abscess or boil. Lesions o this type shown in Fig. 3-17 on the right labia, are usually caused by S aureus. It is important to culture these lesions to rule out the possibility o MRSA. T e organism invades through either an area o damaged skin, a hair ollicle, or a sebaceous gland. As bacteria multiply, a deep cavity containing polymorphonuclear leukocytes and bacteria is ormed. Abscesses can orm anywhere on the body but are most common on the extremities, neck, buttocks, and axillae.

Many individuals with recurrent uruncles are ound to be harboring the causative strain o S aureus in the nares. Rarely, recurrent urunculosis is a sign o an underlying immune de ciency. In the earliest stages, intermittent warm compresses and systemic antibiotics may abort or mature lesions quickly. When lesions are pointed, incision and drainage is the treatment o choice. A carbuncle is a multiloculated abscess that orms when two or more neighboring uruncles become con uent.

Fig ure 3-19

Fig ure 3-20

Streptococcal intertrigo Some in ants develop a sharply demarcated, intensely erythematous and sometimes macerated intertriginous eruption in the neck olds (Fig. 3-19), axillae (Fig. 3-20), or groin area caused by group A β-hemolytic streptococcus. Identical lesions can be caused by in ection with S aureus.

T is is associated with a distinctive oul odor but with an absence o satellite lesions (di erentiating it rom Candida albicans intertrigo). reatment with topical and oral antibiotic therapy is advised. Family members may also su er rom streptococcal pharyngitis or other strep in ections.

25

Se ction 3 . Bacte rial Infe ctions

Fig ure 3-21

Fig ure 3-22

Streptococcal intertrigo Figure 3-21 shows a strep in ection o the popliteal ossae. Note the bee y red, weepy erythema with some crusting at the periphery. T ere are also some pustules at the periphery.

T is young child has a strep in ection in the axillae. One or multiple exural areas may be involved. T ere may be a characteristic odor in the area o involvement.

Fig ure 3-23

Fig ure 3-24

Perianal streptococcal disease Group A β-hemolytic streptococcus is sometimes the cause o perianal in ammation in a child. T is localized in ection is accompanied by pain ul de ecation or pruritus. Examination o the area reveals a brightred erythema surrounding the rectum and oozing rom the in ected area o skin. Diagnosis may be con rmed by perianal swab and culture. S aureus has also been reported to cause a similar clinical picture, and so treatment should be guided by culture results.

Blistering distal dactylitis T is is a distinctive cutaneous in ection that is caused by group A β-hemolytic streptococcus. T e clinical appearance, as shown in Fig. 3-24, is a super cial blister over the anterior at pad o the distal phalanx. One or more ngers may be involved. T e blister uid is culture-positive or the causative bacteria, and treatment consists o incision and drainage along with the appropriate antibiotic by mouth.

26

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 3-25

Fig ure 3-26

Scarlet ever Scarlet ever is a generalized exanthem o childhood, with the highest incidence between the ages o 5 and 15 years. T e cause is in ection (usually o the oropharynx) by group A β-hemolytic streptococcus. T e rash results rom an erythrogenic toxin produced by these bacteria. T e disease begins a er a short incubation period o 2 to 4 days with a pharyngitis, ever, and malaise. T e skin then begins to show a di use punctate erythema with a ne “sandpaper” texture.

T e ace may become ushed but does not become as erythematous as the body. Characteristically, there is pallor around the mouth and the tip o the nose. Erythema is deepest in skin olds, especially the antecubital ossae and the axillary lines, where petechiae in linear arrangement may develop. T ese typical lesions, known as Pastia lines, are seen in Fig. 3-26. ender cervical adenopathy is common.

Fig ure 3-27

Fig ure 3-28

A red pharynx, purulent tonsillitis, and palatal petechiae may be present. During the rst 2 days o the illness the tongue develops a thin white coating with erythema and mild swelling o the papillae (“white strawberry tongue”), as seen in Fig. 3-27.

By the ourth to h day, the white membrane sloughs o revealing prominent papillae on a shiny red tongue as seen in Fig. 3-28.

27

Se ction 3 . Bacte rial Infe ctions

Fig ure 3-29

Fig ure 3-30

Scarlet ever Desquamation o the hands, eet, elbows, and knees occurs during healing.

Occasionally, peeling in these locations may be the sole cutaneous mani estation o a mild, resolving streptococcal in ection.

Fig ure 3-31

Fig ure 3-32

Erysipelas T is is a rare orm o super cial cellulitis caused by group A β-hemolytic streptococci. Erysipelas requently occurs on the ace and presents as a tense, warm, and tender erythematous plaque with a well-demarcated border. In this patient, there is edema o right cheek and multiple erosions. T e patient may be severely ill with ever and local lymphadenopathy. A parenteral antibiotic is o en required in the initial, acute phase.

Invasive group A streptococcal disease Group A β-hemolytic streptococcus may, though rarely, cause severe invasive disease with clinical ndings such as pneumonia, septicemia, necrotizing asciitis, and a toxic shock-like illness. A small proportion o patients with varicella also may develop secondary in ection with streptococcus, leading to severe invasive disease.

28

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 3-33

Fig ure 3-34

Cat-scratch disease T is disease is characterized by the appearance o a papule or pustule at the site o a scratch rom a cat. Within several weeks, enlarged regional lymph nodes (Fig. 3-34) develop and become tender and uctuant. Cat-scratch disease is accompanied by ever and malaise in about one-third o cases.

Localized adenopathy may last rom several weeks to months and then resolves spontaneously. Central nervous system involvement is a very rare but sometimes serious complication. T e disease is caused by Bartonella henselae. reatment with erythromycin, azithromycin, or doxycycline is usually e ective.

Fig ure 3-35

Fig ure 3-36

Erythrasma T is is a airly common condition that is occasionally seen during childhood and increases in requency with age. Lesions occur in the axillae, groin, and toe webs. T e causative organism is a diphtheroid, Corynebacterium minutissimum. Involvement in the axilla, as seen in Fig. 3-36, appears as a well-demarcated brown-to-red plaque. Maceration and scaling

between the toes (Fig. 3-35) is another clinical presentation. A characteristic o the lesion is that it uoresces coral-red under the Wood’s light (3650 Å) because the causative organism produces porphyrins in the stratum corneum. Erythrasma is exceedingly super cial but can become extensive. A 10-day course o oral erythromycin, 250 mg 4 times a day, is the treatment o choice.

29

Se ction 3 . Bacte rial Infe ctions

Fig ure 3-37

Fig ure 3-38

Verruga peruana (Carrion disease) T is in ection, caused by B bacilliformis, is seen in the Peruvian Andes and is transmitted by the Lutzomyia sand y. It also may be seen in travelers rom this area. An acute phase o this in ection, called Oroya ever, is characterized by ever and commonly hemolytic anemia, thrombocytopenia, and elevated liver transaminases. Patients may also have dyspnea, mental status changes, and seizures. Patients with

the eruptive phase develop crops o small nodules that enlarge and develop a vascular appearance. T ese lesions then may ulcerate, bleed, and subsequently heal with brosis over several months. Di erent stages o lesions may coexist. A persistent bacteremia is common. T e pre erred treatment is chloramphenicol, although doxycycline may be e ective. Cutaneous lesions may resemble those o bacillary angiomatosis.

Fig ure 3-39

Fig ure 3-40

Pitted keratolysis T is condition is characterized by numerous shallow, discrete pits on the plantar sur ace o the eet, usually in the weight-bearing areas. Although the condition is asymptomatic, there is usually hyperhidrosis and the eet may be malodorous. Pain ul erosions may occur. T e condition is caused by Micrococcus species. opical clindamycin or topical erythromycin are the treatments o choice.

Actinomycosis T is is a chronic granulomatous disease o worldwide distribution caused by gram-positive obligate parasites that are most closely related to bacteria. Illustrated in Fig. 3-40 is the most common cervico acial orm o the disease. Deep to this super cial neck mass is a ocus o actinomycosis. T e purulent discharge rom the underlying sinus contains yellowish particles, the so-called sul ur granules. T ese granules are colonies o the causative agent, which is usually Actinomyces israelii. Diagnosis is made by culture o the organism on anaerobic media, and the treatment o choice is penicillin.

30

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 3-41

Fig ure 3-42

Cutaneous ef ects o Pseudomonas sepsis Sepsis caused by Ps aeruginosa occurs most commonly in the child with an underlying illness. It is seen in children with immune de ciency due to cancer chemotherapy, in those with malnutrition, and in those with extensive burns. Some o the less speci c cutaneous maniestations, speci cally erythematous macules and petechiae, are shown in Fig. 3-41. Pseudomonas sepsis may also present with discrete small nodules and bullae.

T e classic skin lesion o Pseudomonas sepsis is termed Ecthyma gangrenosum. T is orm characteristically progresses quickly rom a well-circumscribed area o edema to a centrally located blister and then to a gangrenous ulcer with a gray eschar. Multiple lesions in di erent stages o evolution may be present, and the lesions may appear on any body sur ace. Gram stain and culture o tissue scraped rom the base o a blister will be positive or Pseudomonas.

Fig ure 3-43

Fig ure 3-44

Cutaneous ef ects o meningococcemia T e early treatment o meningococcemia with appropriate parenteral antibiotics can o en be li esaving. Cutaneous mani estations o this disease may provide the single most important diagnostic clue in the acutely ill child. T e most common ndings on the skin are petechiae and purpura. T e small petechial lesion shown in Fig. 3-43 develops early in the course o the disease, and a scraping yields gram-negative diplococci on both Gram stain and culture. Subsequently, the lesions arise in additional crops, enlarge, and coalesce.

T e numerous ecchymoses shown in Fig. 3-44 are the result o this rapidly ongoing process. T ese areas may become necrotic and develop eschars. Other cutaneous mani estations, occurring subsequently, include peripheral gangrene and purpura ulminans, with con uent areas o necrosis o the skin. T ese are the result o vasospasm, shock, and a consumption coagulopathy. A de ciency in protein C may be another cause o this process.

31

Se ction 3 . Bacte rial Infe ctions

Fig ure 3-45

Fig ure 3-46

Cutaneous ef ects o gonococcemia In ection with Neisseria gonorrhea may mani est as a purulent conjunctivitis in the newborn or as a genital discharge in the sexually abused child or sexually active adolescent. Disseminated gonococcal in ection, as shown in Fig. 3-45, is quite rare. Gonococcemia presents with a migratory polyarthralgia or septic arthritis and ever.

T e skin lesions are ew, are o en located on extremities, and may overlie the involved joints. Initially, there are small erythematous macules that progress to papules. T ese tender lesions may develop a small vesicle and then a gray, umbilicated center. Rarely, bullae, petechiae, and larger hemorrhagic lesions are also seen. Figures 3-45 and 3-46 show the macules and necrotic papules that are typical o disseminated gonococcal in ection.

SECTION

4 Spirochetal, Protozoal, Mycobacterial, and Rickettsial Diseases

34

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 4-1

Fig ure 4-2

Congenital syphilis In utero in ection by the spirochete Treponema pallidum can occur a er the 16th week o gestation. Intrauterine disease, especially during early pregnancy, may result in spontaneous abortion or in a severely a ected in ant. Severe disease that is present at birth presents with hepatosplenomegaly, ascites, meningoencephalitis, and severe anemia. Osteochondritis is the most characteristic bone change. T e cutaneous ndings in severe congenital syphilis include bullae, pustules, macules, and papules.

Fissuring and peeling o the skin are also characteristic. he palms, soles, and periori icial skin are sites o predilection. Syphilitic rhinitis, with a copious and bloody nasal discharge, is an associated nding. I in ection occurs late in pregnancy, signs and symptoms may be delayed or several weeks. In these cases, diagnosis is usually made on the basis o a positive syphilis serology in mother and in ant. I the disease is allowed to progress, rhinitis, cutaneous macules, and mucous patches may be the presenting signs.

Fig ure 4-3

Fig ure 4-4

Acquired syphilis Unlike congenital syphilis, acquired syphilis in in ants, children, and adolescents ollows the classic course o syphilis in adults. Such an in ection in a child should be assumed to be the result o sexual abuse. T e rst event in the development o syphilis is a dark- eld positive chancre at the portal o entry o the treponeme. Shortly therea er, serologic tests or syphilis become positive.

Secondary syphilis usually develops 6 to 8 weeks a er the appearance o the chancre. Malaise, low-grade ever, myalgias, and lymphadenopathy are accompanied by a wide variety o cutaneous mani estations. T e lesions shown in Fig. 4-3 are condylomata lata around the rectum. Note the moist papules and plaques. Figure 4-4 shows the most common presentation: copper-colored papulosquamous lesions, most commonly on the palms and soles.

Se ction 4 . Spiroche tal, Protozoal, Mycobacte rial, and Ricke tts ial Dis e as e s

35

Fig ure 4-5

Fig ure 4-6

Acquired syphilis Sometimes the eruption resembles pityriasis rosea, as seen in Fig. 4-5. Other cutaneous mani estations o secondary syphilis include papular lesions, pustules, nodules, and plaques.

Mucous patches are a common mani estation o acquired syphilis, and appear as white slightly raised plaques on an erythematous base with a serpentine, well-de ned border.

Fig ure 4-7

Yaws his is a nonvenereal treponematosis that is caused by T pertenue. It is endemic in areas o Central and South America, A rica, and Southeast Asia. T e disease is acquired by physical contact, and the majority o cases occur during childhood. An ulceration occurs at the site o the primary inoculation. Secondary lesions are cutaneous nodules or moist or hyperkeratotic plaques; they appear within several weeks and resolve spontaneously. Recurrence o latent disease, with gummata o the skin and bones, may occur many years later.

36

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 4-8

Fig ure 4-9

Erythema migrans (Lyme disease) Lyme disease is caused by the spirochete Borrelia burgdorferi and is transmitted by the pinhead-sized Ixodes ticks. T e illness is endemic in large areas o the continental United States. T e early cutaneous mani estation, termed erythema migrans, is shown in Figs. 4-8 and 4-9. It consists o an expanding annular lesion around the original tick bite.

Satellite areas o involvement may also be present. Multiple lesions o erythema migrans may represent early disseminated Lyme disease as seen in Fig. 4-11.

Fig ure 4-10

Fig ure 4-11

Erythema migrans (Lyme disease) Pruritus or burning may be present at the site o the lesion, and the rash may be accompanied by ever, malaise, and regional lymphadenopathy. T e systemic mani estations o Lyme disease include neurologic dys unction (eg., Bell’s palsy), cardiac conduction abnormalities, and arthritis.

Early antibiotic therapy or the typical skin lesion will o en prevent the development o the more serious and long-lasting systemic illness. Serologic testing is o some value in diagnosis but results may be negative early on, especially in the absence o neurologic or joint symptoms.

Se ction 4 . Spiroche tal, Protozoal, Mycobacte rial, and Ricke tts ial Dis e as e s

37

Fig ure 4-12

Fig ure 4-13

Lepromatous leprosy Leprosy, or Hansen disease, is a chronic multisystem disease that is caused by Mycobacterium leprae, an acid- ast bacillus. T e highest incidence o the disease is in areas o South America, A rica, and Asia. It is not rare in children. T e clinical mani estations o this illness depend on the host response to in ection. At one end o the spectrum is lepromatous leprosy (LL), which represents a diminished host response to the leprosy bacillus. Cutaneous lesions in this orm o the

disease vary. Macular lesions are symmetrically distributed hypopigmented and erythematous patches. When widespread involvement occurs, the lesions may be di cult to di erentiate rom normal skin. T e lesions pictured here are more in ltrative. Nodular lesions o the earlobe, as shown in Fig. 4-12, are particularly common in LL. Annular plaques and papules (Fig. 4-13) may also be present. LL is the orm most likely to cause widespread nerve damage and ocular disease.

Fig ure 4-14

Fig ure 4-15

Tuberculoid leprosy Patients with tuberculoid leprosy (classi cation ) are those who have the strongest immune response to chronic in ection. T e cutaneous lesions are typically sharply demarcated plaques with a rm raised border. Varying degrees o erythema, hypopigmentation, and anesthesia may be present. Note the large, sharply demarcated areas o decreased pigmentation in Fig. 4-14, and the raised border o the lesion in Fig. 4-15.

In early disease, the loss o sensation and normal sweating in involved areas may be di cult to detect, and the lesions can easily be con used with vitiligo and pityriasis alba. tends to remain a localized disease and spontaneous resolution may occur. Nerve damage tends to be limited to one or two nerves. Combinations o ri ampicin, clo azimine, and dapsone are required in many cases.

38

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 4-16

Fig ure 4-17

Dimorphous leprosy T e terms borderline and dimorphous leprosy are used to re er to patients whose disease shows eatures o both the lepromatous and tuberculoid orms. Patients with dimorphous leprosy (classi ed as BL, BB, or B ) have varied skin lesions, including the plaques and annular lesions shown in Figs. 4-16 and 4-17. T ey o en su er widespread and rapidly progressive nerve damage. Knowledge o the proper treatment o leprosy must include an understanding o the acute

exacerbations that may accompany therapy. reatment o nonlepromatous leprosy with antibiotics may induce erythema and edema o the skin lesions, accompanied by rapid damage to the peripheral nerves (type I reaction). ype II reaction, also termed as erythema nodosum leprosum, is most common in patients who have LL. It consists o pain ul dermal nodules accompanied by ocular disease, peripheral neuropathies, and a wide variety o constitutional symptoms.

Fig ure 4-18

Fig ure 4-19

Primary complex of tuberculosis in the skin A child who has an area o injury to the skin may develop a primary complex in that location when exposed to someone with active tuberculosis. A er an incubation period o 1 to 3 weeks, a red papule develops and evolves into a nodule or plaque with ulceration (tuberculous chancre). T e tuberculous ulcer is accompanied by regional lymphadenopathy. T is orm o cutaneous tuberculosis is a sel -limited disease, and a slow healing with scar ormation occurs.

Scrofuloderma T e skin lesions o scro uloderma result rom extension o tuberculosis rom areas o in ection in the bones, joints, muscle, or most commonly lymph nodes. T e lesion begins as a nodule and evolves into an ulcer with draining sinuses. Figure 4-19 shows a case o tuberculous cervical lymphadenitis, with the channeling o sinuses to the skin sur ace. T is orm o cutaneous in ection by M tuberculosis is particularly common in children.

Se ction 4 . Spiroche tal, Protozoal, Mycobacte rial, and Ricke tts ial Dis e as e s

39

Fig ure 4-20

Fig ure 4-21

Lupus vulgaris T is is a progressive orm o cutaneous tuberculosis that results rom either primary inoculation or the hematogenous spread o M tuberculosis. It is seen in patients who are very sensitive to the organism. Favored locations are the central ace, earlobes, and other parts o the head and neck.

T e lesions may be papules, nodules, or plaques, and the color is o en described as “apple jelly.” Older lesions, as shown in Fig. 4-21, are brownish annular plaques; the area o central clearing represents an attempt at healing.

Fig ure 4-22

Fig ure 4-23

Tuberculosis cutis verrucosa T is orm o cutaneous tuberculosis caused by Mycobacterium tuberculosis or Mycobacterium Bovis results rom exogenous rein ection o an already tuberculinsensitive individual. Although this rein ection can occur rom contact with tuberculous tissue, children can develop this a er contact with tuberculous sputum. T ere is most likely a history o prior injury to the oot.

Lesions most commonly occur in children on the lower extremities. When the lesions are located on the hands or eet, they tend to develop a distinctly verrucous sur ace, beneath which is inf ammation. T is may start with a small warty growth which slowly progresses peripherally. T e resultant large warty plaque may develop central scarring, as is seen in Fig. 4-22. Purulent material may be expressed.

40

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 4-24

Fig ure 4-25

Lichen scrofulosorum T is term describes a condition that consists o grouped lichenoid papules on the trunk. It occurs in children who have active tuberculosis in other locations. T e individual lesions are f at-topped ollicular papules and are either erythematous or f esh-colored. T e condition is asymptomatic and ollows a benign course to spontaneous resolution. Recurrences are common.

Papulonecrotic tuberculid Clinically this condition is highly stereotypic. It consists o dusky red match head- to pea-sized sterile papules that arise in symmetrical crops on the extensor aspects o the extremities, usually on the elbows and knees. Occasionally the buttocks are also involved. T e condition is asymptomatic and heals spontaneously with varioli orm scars. Polymerase chain reaction (PCR) ampli cation has detected tuberculosis DNA in these lesions.

Fig ure 4-26

Fig ure 4-27

Infection with atypical mycobacteria T ere are a number o cutaneous in ections that are caused by mycobacteria other than M tuberculosis or M leprae. Illustrated here is in ection by M marinum. T is organism is known to contaminate sh tanks and swimming pools, and the cutaneous in ection is sometimes termed swimming pool granuloma. In act, the case shown in Fig. 4-26 was traced to a sh tank and that in Fig. 4-27 to a swimming pool.

T e lesions usually appear at a point o trauma and the hands, eet, elbows, and knees are particularly common locations. A er an incubation period o 2 to 6 weeks, the lesions evolve rom small papules into single or grouped violaceous nodules. Satellite lesions may be present. Biopsy o a mature lesion reveals a caseating granuloma, and acid- ast organisms within histiocytes can sometimes be identi ed. Diagnosis can also be made by culture o biopsied material.

Se ction 4 . Spiroche tal, Protozoal, Mycobacte rial, and Ricke tts ial Dis e as e s

41

Fig ure 4-28

Fig ure 4-29

Infection with atypical mycobacteria Figure 4-28 is another instance o a granulomatous process caused by a Mycobacterium other than that o tuberculosis. In this case, the organism was M avium-intracellulare. T e lesion resembles that o tuberculosis cutis colliquativa. T e causative organism was cultured rom the discharge that issued rom sinuses emerging rom in ected lymph nodes. T e patient was positive in reaction to conventional tuberculin and to tuberculins derived rom the atypical mycobacteria.

Amebiasis cutis Entamoeba histolytica, the cause o intestinal and cutaneous amebiasis, extends to the skin rom intestinal in estation. Discharging rom the ulcerous process in the lower part o the gut, viable amoebas contaminate and may in ect the perianal region. When the in ection is seen in an active state, the process is a continually creeping, pain ul ulceration that has little tendency to heal spontaneously. When the in ection is healed, the clinical appearance is a scar, as shown in Fig. 4-29.

Fig ure 4-30

Fig ure 4-31

Leishmaniasis T e cutaneous orm o leishmaniasis is caused by a protozoan (Leishmania tropica) that is transmitted by the bite o a sandf y (o the genus Phlebotomus) in certain endemic regions (the Middle East, China, A rica, India, the ormer Soviet Union). T e primary process in the skin starts as a macule o erythema, evolves into a papule (Fig. 4-30), and then develops into a granulomatous or ulcerous process (Fig. 4-31). T e lesion takes up to 1 year to heal spontaneously in the orm o a vaccini orm scar.

Systemic or visceral leishmaniasis (kala-azar) is caused by the related protozoan L donovani. T is orm is characterized by ever, anemia, leukopenia, emaciation, and severe hepatosplenomegaly. T e skin develops a grayish pigmentation, usually on the ace. Finally, mucocutaneous leishmaniasis caused by L braziliensis, is characterized by destructive mucosal lesions, and is endemic in some areas o Latin America.

42

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 4-32

Fig ure 4-33

Rickettsialpox T is sel -limited ebrile illness is caused by Rickettsia akari. It is transmitted to man by a mite that is an obligatory parasite o the common house mouse. One to two weeks a er exposure, a nodule develops at the point o entry o a parasitized mite. T e nodule becomes vesicular and then crusted with a black eschar (the pock, seen well developed on the neck in Fig. 4-32). Regional lymphadenopathy is usually present.

Within several days, the patient develops intermittent ever, photophobia, headache, myalgia, and lassitude. wo to three days later, a generalized exanthem, consisting o discrete macules that quickly become papular, appears (Fig. 4-33). Firm vesiculopustules arise atop the papules and these subsequently orm crusts. T e eruption lasts or 1 to 2 weeks. T e disease must be treated on clinical suspicion. Serologic tests or antibodies and biopsy or culture, direct f uorescent antibody, and PCR can all be used to con rm the diagnosis.

Fig ure 4-34

Fig ure 4-35

Rocky Mountain spotted fever his disease is caused by Rickettsia rickettsii and is transmitted by a number o di erent ticks. Despite its geographical title, Rocky Mountain spotted ever is present in many locations throughout the United States and the entire Western Hemisphere. A er in ection by tick bite, there is an incubation period o 2 to 14 days. T e abrupt onset o the disease includes severe headache, ever, chills, arthralgia, and myalgia. A er 2 to 3 days o these constitutional symptoms, erythematous macules erupt on the wrists, hands, orearms, legs, and ankles, as

seen in Figs. 4-34 and 4-35. Lesions then spread to the palms and soles and the trunk. T e macules originally blanch with pressure but soon become purpuric and even necrotic. T e disease causes a severe vasculitis and complications include disseminated intravascular coagulation, hemorrhage into the gastrointestinal and urinary tracts, and cardiovascular collapse. T e high atality rate is markedly reduced by prompt antibiotic therapy and the disease must be treated immediately upon clinical suspicion. PCR or immunohistochemical staining o a skin lesions can con rm the diagnosis.

SECTION

5 Viral Diseases

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Fig ure 5-1

Fig ure 5-2

Molluscum contagiosum T is condition is a benign viral in ection that appears as crops o discrete, slightly umbilicated, esh-colored, or shiny papules. It is extremely common among children and may be seen in several children within a amily. T e lesions may become in amed i traumatized or in ected and sometimes become in amed spontaneously as they resolve.

T e lesions tend to be grouped, and the average size o a lesion is 2 to 3 mm in diameter and height. T e trunk, ace, genitalia, and intertriginous areas are the most common sites o in ection. Pruritus is an occasional symptom and an eczematous eruption may develop in the area o the molluscum.

Fig ure 5-3

Fig ure 5-4

Molluscum contagiosum T is viral in ection is sel -limited, but treatment is o en required because o discom ort or out o concern or appearance. reatment should be individualized to the age and extent o involvement in each patient.

As the in ection is sel -limited, observation is an acceptable option. In the cooperative patient, destruction o lesions with curettage or light cryotherapy may be attempted or treatment o limited lesions. Some dermatologists treat this disorder with the o ce application o topical cantharidin. T e child with numerous lesions poses a particular therapeutic challenge.

45

Se ction 5 . Viral Dis e as e s

Fig ure 5-5

Fig ure 5-6

Molluscum contagiosum Occasionally, a lesion o molluscum contagiosum may grow to as large as 3 cm in diameter. wo photos o such “giant mollusca” are shown in Figs. 5-5 and 5-6. T e diagnosis is usually suggested by the presence o more typical,

smaller lesions on adjacent or distant skin sur aces. Note the presence o a central umbilication in Fig. 5-5. reatment is by surgical removal when possible.

Fig ure 5-7

Fig ure 5-8

Molluscum contagiosum When molluscum contagiosum appear in the groin or especially the intergluteal cle area, they may be misdiagnosed as warts. Molluscum in the intergluteal cle area may appear like “ eshy” skin tags, and upon close examination that can be aided by magni cation, a central umbilication can be seen. I the diagnosis is in question, a biopsy would yield the diagnosis.

Some patients with molluscum contagiosum will develop scarring rom this viral in ection. Large and even small molluscum may scar even without any treatment. Figure 5-8 shows a patient who developed scarring without any treatment given or the molluscum.

46

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Fig ure 5-9

Fig ure 5-10

Molluscum contagiosum dermatitis Many children with molluscum contagiosum in ection develop an eczematous, pruritic dermatitis surrounding the af ected area o involvement. Low

potency topical corticosteroids can alleviate the dermatitis. At times, the lesions in the involved area may have to be treated in order to permanently clear the dermatitis.

Fig ure 5-11

Fig ure 5-12

Molluscum contagiosum id reaction Some patients may develop a hypersensitivity eruption as a result o the immune response to the virus. T is eruption avors the extremities, particularly the elbows (Fig. 5-11) and knees, with skin colored to erythematous papules and grouped papulovesicles. T e buttocks

may also be involved. In amed molluscum is seen elsewhere. Note the in amed mollusum on the abdomen in Figs. 5-11 and 5-12. T e papules on the elbow and hand in Fig. 5-12 are related to the id reaction and are not in amed molluscum.

47

Se ction 5 . Viral Dis e as e s

Fig ure 5-13

Fig ure 5-14

Verruca vulgaris T e common wart is a benign growth caused by localized in ection with one o the many types o human papillomavirus. T ese small DNA viruses are part o the papovavirus group. Warts are especially common among children and adolescents and may occur on any mucocutaneous sur ace. T e hands are a particularly requent location.

T e typical wart is a rough-sur aced nodule that may be either lighter or darker than the surrounding skin. Lesions involving the proximal and lateral nail olds are common and present particular treatment di culties.

Fig ure 5-15

Fig ure 5-16

Verruca vulgaris Gentle cryotherapy or topical salicylic acid preparations may be o value in the treatment o warts. However, severe warts such as those shown in Fig. 5-15 may require additional therapies, such as injection with intralesional candida antigen.

Figure 5-16 illustrates multiple warts located on the palmar surace o the hand. In general, treatment must be individualized to the age and cooperative abilities o the patient and to the size and location o the warts.

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Fig ure 5-17

Fig ure 5-18

Verruca vulgaris Following the application o cantharidin or liquid nitrogen, blister ormation may result. Upon resolution o the blister, a ring wart may develop, as seen in Fig. 5-17.

Multiple ring warts are seen in Fig. 5-18.

Fig ure 5-19

Fig ure 5-20

Verruca vulgaris Although the hands are the most common location or warts in children, they may occur on almost any part o the skin sur ace.

Figure 5-20 illustrates the appearance o multiple clustered warts on the knee.

49

Se ction 5 . Viral Dis e as e s

Fig ure 5-21

Fig ure 5-22

Plantar warts Plantar warts appear as at areas o rm hyperkeratosis on the soles o the eet. Lesions that occur at points o pressure may be associated with severe pain on walking. Figure 5-21 illustrates solitary and multiple plantar warts. Note the obliteration o skin markings, which does not occur in a callus. T e lesions in Fig. 5-22 on the heel are numerous and mosaic. reatment o plantar warts requires perseverance on the part o both patient and physician.

Attempts at a rapid cure may result in scarring. One practical method involves the daily application o salicylic acid plasters or liquid salicylic acid preparations along with repeated paring o the necrotic sur ace o the wart. T e success o this routine may be hastened by gentle cryotherapy.

Fig ure 5-23

Fig ure 5-24

Fili orm warts T e sur aces o common warts are in uenced by their position on the body. In general, they have a rough sur ace. On hands, the sur aces o warts are doomed rom wear and tear, so that troughs and crests are shallow; on soles, they are at and smooth rom the weight o the body, but where they are undisturbed, common warts tend to grow with mbria or ngerlike projections. T ey are then called f li orm or digitate warts.

T e ace and scalp are the most common sites where warts grow in this ashion Fili orm warts may also occur on the lips as shown in Fig. 5-24.

50

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 5-25

Fig ure 5-26

Verruca plana Plane, or at, warts may be caused by several types o the human papillomavirus. T ey are common in children. T e lesions are slightly raised, esh-colored papules, and usually 2 to 4 mm in diameter.

T e ace and hands are the most requent locations or these multiple small warts. T e lesions may be discrete or con uent, and a linear array o at warts, as shown in Fig. 5-26, may result rom autoinoculation in a scratch.

Fig ure 5-27

Fig ure 5-28

Condyloma acuminatum Warts with this “cauli ower” appearance on the labia (Fig. 5-27), penis, or around the rectum (Fig. 5-28) are termed condylomata acuminata. T e presence o lesions o this type in a child should prompt the physician to consider the possibility o sexual abuse, although the true incidence o this association is not known. In very young children, perinatal transmission is probably the most common cause.

In some cases, as well, condylomata acuminata may be the result o innocent contact with another in ected individual. T e use o topical products containing podophyllum or imiquimod are ef ective treatments.

51

Se ction 5 . Viral Dis e as e s

Fig ure 5-29

Epidermodysplasia verruci ormis T is rare amilial disease usually has its onset during childhood. Patients develop numerous at warts, initially involving the ace and upper trunk. A number o human papillomavirus types have been implicated. As the lesions tend toward con uence, they may mimic the appearance o tinea versicolor. Most patients with epidermodysplasia verruci ormis have depressed cell-mediated immunity, and they are also at risk or squamous cell carcinoma. A similar presentation may be seen in children with HIV in ection.

Fig ure 5-30

Fig ure 5-31

Neonatal herpes simplex Neonatal herpes simplex in ection is acquired during passage through an in ected birth canal. T e cutaneous involvement may be as extensive as shown in Fig. 5-30, or it may be subtle. T e scalp is the most common site or the typical clustered vesicles.

Because o the very high requency o concurrent disseminated disease, cutaneous herpes simplex in the newborn must be considered a pediatric emergency. Early treatment with an intravenous antiviral agent minimizes complications and of ers the in ant the best possibility o survival.

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Fig ure 5-32

Fig ure 5-33

Herpes simplex Herpes simplex in ection o the ngers may occur in in ants and young children. T e rst episode may accompany herpetic gingivostomatitis.

Herpetic whitlow Herpetic whitlow presents with pain or paresthesia and with erythema and vesicle ormation. T e original in ection is sometimes misdiagnosed as a bacterial or candida paronychia, but subsequent recurrences lead the practitioner to the correct diagnosis.

Fig ure 5-34

Fig ure 5-35

Herpetic gingivostomatis Primary in ection with herpes simplex may cause a severe gingivostomatitis that is severely painul and inter eres with eating and drinking. reatment consists o topical analgesics, supportive care, and oral or intravenous acyclovir.

Recurrent herpes simplex Recurrent herpes simplex in ections can occur in a wide variety o locations, including, as shown in Fig. 5-35, the ear. T e original episode could easily be mistaken or a bacterial in ection.

53

Se ction 5 . Viral Dis e as e s

Fig ure 5-36

Fig ure 5-37

Herpes simplex, recurrent Following an episode o primary herpes simplex, the virus may remain dormant within a nerve ganglion. Represented in Fig. 5-36 and Fig. 5-37 are lesions on the ace showing a less common location serving to illustrate that recurrent herpes simplex may occur on any area o the skin.

A number o triggering actors including ebrile illness, emotional or physical stress, excessive sun exposure, and trauma may cause the virus to replicate and spread to the skin sur ace.

Fig ure 5-38

Fig ure 5-39

Herpes simplex, recurrent Patients with recurrent disease will o en experience prodromal pain or paresthesia in the involved area. Most episodes last rom 7 to 10 days. Figure 5-38 shows lesions on the knee. T is is a less common location, serving to illustrate that recurrent herpes simplex may occur on any area o the skin.

Genital herpes simplex Herpes simplex in ection in the genital region is a sexually transmitted disease. It is common in the sexually active adolescent as shown in Fig. 5-39. In the young child, genital herpes simplex should create a high index o suspicion or sexual abuse. T is gure illustrates the multiple pain ul erosions that may occur in primary in ection. Regional lymphadenopathy and ever may also be present. Recurrences o genital herpes are very common but are generally much milder in both extent and duration than the primary disease.

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Fig ure 5-40

Fig ure 5-41

Eczema herpeticum (Kaposi varicelli orm eruption) Disseminated cutaneous herpes simplex in ection is a serious complication o atopic dermatitis. It may also occasionally occur in individuals with other dermatologic disorders. Patients rapidly develop numerous umbilicated vesicles in the areas o eczematous involvement. Children with eczema herpeticum o en become seriously ill with high ever. Bacterial superin ection and sepsis can be a complication, and the disease is occasionally atal.

T e patients pictured here developed involvement o the entire skin sur ace. T e lesions in Fig. 5-40 are typical umbilicated vesicles, and the lesions in Fig. 5-41 are equally extensive and are beginning to crust. Skill ul and attentive supportive treatment is required in order to maintain uid and electrolyte balance and prevent superin ection. reatment with intravenous acyclovir hastens clinical improvement and is recommended or children with extensive or rapidly increasing involvement.

Fig ure 5-42

Fig ure 5-43

Varicella Chickenpox is caused by a virus o the herpes group. T e disease is highly contagious and is spread by droplet or direct contact. T e incubation period or chickenpox ranges rom 11 to 21 days. Prodromal symptoms consist o low-grade ever, headache, anorexia, and malaise. On the ollowing day, the characteristic rash begins to appear. T e lesions evolve rom erythematous macules to orm small papules. Quickly, a clear vesicle arises on this erythematous base.

T e classic lesion o chickenpox has been poetically described as a “dewdrop on a rose petal”. Over the next several days, the vesicles rupture and then crust. T e rash begins on the chest and back and spreads centri ugally to involve the ace, scalp, and the extremities. New lesions o chickenpox arise in crops over a period o several days. Since crops o macules, papules, vesicles, and crusts are successive and overlapping, one sees lesions at all stages o development in given locations. Itching is a symptom and may be severe.

55

Se ction 5 . Viral Dis e as e s

Fig ure 5-44

Fig ure 5-45

Varicella Figure 5-44 depicts a case o chickenpox o unusual severity. T e lesions tend to be large blisters and to become hemorrhagic. Cases o this type are more likely to occur in children with reticuloendothelial malignancies or immunologic de ects, or in those under immunosuppressive or prolonged corticosteroid therapy. T is patient had scarlet ever 2 weeks prior to the varicella outbreak.

Lesions on the oral mucous membranes, which are common, are pictured in Fig. 5-45. T ey are most common on the palate and quickly evolve into small, super cial erosions.

Fig ure 5-46

Fig ure 5-47

Varicella Other, less requent, sequelae include thrombocytopenia, purpura ulminans, and postin ectious encephalitis. Finally, a signi cant number o cases o Reye syndrome occur a er chickenpox, and aspirin should there ore not be used as an antipyretic.

Involvement o varicella in in the scalp is shown in Fig. 5-47. Complications o chickenpox may include varicella pneumonia, myocarditis, and hepatitis. Secondary bacterial in ection o the skin lesions is by ar the most common complication o chickenpox.

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Fig ure 5-48

Fig ure 5-49

Varicella In rare cases o chicken pox, lesions occur primarily or exclusively in sun-exposed areas. Figure 5-48 shows an example o photo-related varicella, with a concentration o lesions on the sun-exposed areas o the mid-upper chest.

Congenital varicella syndrome On rare occasions, the in ants o women who develop chickenpox during the rst trimester o pregnancy are born with congenital de ects. T e congenital varicella syndrome is characterized by asymmetrical arm or leg lengths, ocular abnormalities, microcephaly, and intrauterine growth retardation. Cutaneous scarring in a dermatomal distribution is particularly characteristic o the constellation o birth de ects caused by the varicella zoster virus.

Fig ure 5-50

Fig ure 5-51

Neonatal varicella Maternal chickenpox during several days be ore or a er the birth o a child may lead to a signi cant health risk or the in ant. T e newborn who is exposed to varicella without the bene t o transplacental maternal antibody may go on to develop severe disseminated in ection with pulmonary

and hepatic involvement. T is disease may sometimes be atal. Pictured here are newborns with numerous typical chickenpox lesions. T e hemorrhagic lesions that may be part o neonatal varicella are not present in these cases.

57

Se ction 5 . Viral Dis e as e s

Fig ure 5-52

Fig ure 5-53

Herpes zoster Herpes zoster, or shingles, is an acute eruption characterized by vesicles and small bullae. Lesions are usually unilateral and con ned to a single dermatome. T e cause o herpes zoster is the varicella zoster virus. A er an initial episode o chickenpox, the virus lies dormant in the dorsal root or cranial nerve ganglia. Reactivation o the virus, usually years later, leads to the typical eruption.

Herpes zoster is a relatively rare disease in children and is seen more requently in those young people who had chickenpox at a very early age. Localized pain may precede the onset o the rash, but postherpetic neuralgia is unusual in childhood. Children with lesions around the eye or on the tip o the nose may have involvement o the nasociliary nerve and should be observed care ully or ocular involvement. Children with involvement o the ear, shown in Fig. 5-53, may develop severe otalgia and when associated with acial paralysis it is known as Ramsay Hunt syndrome.

Fig ure 5-54

Fig ure 5-55

Herpes zoster T e child with herpes zoster may, in the normal course o the illness, develop several chickenpox-like lesions outside o the involved dermatome. However, rapid and widespread evolution o such lesions—generalized herpes zoster—is a problem o greater concern. Hematogenous spread o the viral in ection in this ashion may be an indication o an underlying immune de ciency.

In ants born to mothers who developed primary varicella in ection during pregnancy may likewise have been in ected during etal li e. T ese in ants may develop herpes zoster without a clinical history o previous varicella. Associated with this may be a temporary paresis o the af ected area.

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Fig ure 5-56

Fig ure 5-57

Hand- oot-mouth disease T is common and benign viral disease o childhood is usually caused by the A16 strain o coxsackievirus, although other strains o the same virus have been implicated. It most o en occurs in late summer and early all. T e prodrome consists o low-grade ever and malaise.

Shortly therea er, vesicular lesions arise on the so palate (Fig. 5-56), tongue, buccal mucosa, and uvula. T e lips are usually spared. Occasionally, these lesions may be pain ul and cause some di culty in eating. T e cutaneous lesions develop 1 or 2 days a er those in the mouth. T ey consist o asymptomatic round or oval vesiculopustules that evolve into super cial erosions. T e edges o the palms (Fig. 5-57) and soles (Fig. 5-58) are a avored location.

Fig ure 5-58

Hand- oot-mouth disease T e dorsa o the hands and eet may also be involved. T ere is sometimes an accompanying macular and papular eruption on the buttocks. T e eruption lasts rom 7 to 10 days and no therapy is required. Although culture o the virus is possible, the diagnosis o hand- oot-mouth disease is usually made on clinical grounds.

59

Se ction 5 . Viral Dis e as e s

Fig ure 5-59

Fig ure 5-60

Atypical hand- oot-mouth disease Since 2008, outbreaks o an atypical orm o hand- oot-mouth disease, caused by coxsackie virus A6, have been documented in countries throughout the world. T e development o skin lesions is o en accompanied by ever, chills, malaise, and diarrhea. Perioral lesions, along with involvement inside the mouth, are common, and, as seen in Fig. 5-59, may be con used with impetigo.

Figure 5-60 illustrates the development o numerous lesions on the palms o the hands. Although hand, oot and mouth are sites o predilection or this atypical orm, lesions tend to be more widespread, and, in children with atopic dermatitis, occur in areas o involvement o the eczema itsel , such as the antecubital and popliteal ossa (“eczema coxsackieum”).

Fig ure 5-61

Fig ure 5-62

Atypical hand- oot-mouth disease Vesicles and bullae, some with central umbilication, are requently seen in this variant o hand- oot-mouth disease. T e lesions may be tender.

T e bullae in atypical hand- oot-mouth disease rapidly evolve into super cial erosions, and, in severe cases, this may involve a large part o the skin sur ace. In the resolving phase o the disease, it is not uncommon or children to shed ngernails and toenails.

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Fig ure 5-63

Fig ure 5-64

Complications o vaccinia T e worldwide eradication o smallpox has eliminated the need or vaccination with the modi ed cowpox virus. Over the many years when this medical practice was necessary, a number o benign and some more serious dermatologic complications were observed. T e sequelae o smallpox vaccination are as ollows: 3 to 4 days a er inoculation, there develops an area o erythema and edema at the injection

site (usually an arm or thigh); this rapidly evolves into edema, vesiculation, ulceration, crusting, and scar ormation over a period o about 3 weeks. For most children, ever, malaise, and local discom ort are the major side ef ects. T e complications o a severe local reaction (Fig. 5-63) and o a generalized eruption (Figs. 5-64 and 5-65) are illustrated here.

Fig ure 5-65

Fig ure 5-66

Complications o vaccinia Still another complication o vaccinia vaccination was accidental autoinoculation on an undesirable site while the primary pock was in progress. Autoinoculation then resulted in a new lesion that began in 1 day (a reaction time) and rapidly repeated the whole process. T e seriousness o such accidents was site-speci c: on the ace, particularly around the eyes, blindness, and elsewhere on exposed skin, cosmetic de ects, were serious consequences.

Eczema vaccinatum Disseminated vaccinia was the most dreaded complication o smallpox vaccination. Like eczema herpeticum, eczema vaccinatum occurred in children with underlying eczematous disorders. It was more serious because o the severity o the acute illness, the tendency toward scarring, and a higher incidence o mortality. Figure 5-66 suggests that the scarring rom eczema vaccinatum can be dis guring.

61

Se ction 5 . Viral Dis e as e s

Fig ure 5-67

Fig ure 5-68

Exanthem subitum (roseola) Roseola is a common childhood illness that is now known to be caused by human herpes virus 6. Most cases occur during the rst year o li e. T e disease is characterized by 3 to 5 days o high ever accompanied by minimal constitutional symptoms. Pharyngitis, periorbital edema, and cervical adenopathy may be present and ebrile seizures sometimes occur.

T e disappearance o ever is accompanied by the onset o rash. T e typical eruption is macular and papular, and it is concentrated on the neck and trunk. T e eruption clears in hours or at most a day or two. Note the light pink macules on the in ant pictured in Fig. 5-68.

Fig ure 5-69

Fig ure 5-70

Unilateral laterothoracic exanthem T is condition o unknown etiology begins as an eruption in a peri exural area, the axillary old being the most common. Other exural areas including the inguinal old and popliteal areas may be involved initially. T e eruption then spreads unilaterally in an asymmetric ashion, at times involving an entire side o the body.

T e eruption may then generalize to the rest o the body, typically maintaining predominance on the original side. Clinically, the eruption is scarlatini orm or eczematous in nature. About 50 percent o patients may develop pruritus. Spontaneous resolution usually occurs in 4 to 6 weeks.

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Fig ure 5-71

Fig ure 5-72

Papular acrodermatitis o childhood (Gianotti-Crosti syndrome) T is condition is characterized by the eruption o rm erythematous papules on the extremities, cheeks, and buttocks. T e papulonodular lesions may become con uent in some areas, as illustrated in Figs. 5-71 and 5-72.

T is disease was originally seen in children with anicteric hepatitis B in ection in Europe. Since that time, this syndrome has been ound to occur primarily in association with Epstein-Barr virus in ection, although other causes include coxsackie, parainuenza, poliovirus, vaccinia, β-hemolytic streptococcus, and bacille Calmette-Guérin (BCG).

Fig ure 5-73

Fig ure 5-74

Papular acrodermatitis o childhood (Gianotti-Crosti syndrome) Figures 5-73 and 5-74 both illustrate lesions occurring on the legs, with relative sparing o the popliteal ossae.

T e papules in Gianott-Crosti syndrome are, at rst, brightly erythematous and extremely rm to the touch. T e disease is sel -limiting, lasting 6 to 8 weeks.

63

Se ction 5 . Viral Dis e as e s

Fig ure 5-75

Fig ure 5-76

Congenital cytomegalovirus in ection Congenital in ection with cytomegalovirus, a DNA virus o the herpes group, results in disease o varying severity. While some neonates are completely asymptomatic, severely af ected in ants display intrauterine growth retardation, microcephaly, and hepatosplenomegaly. T e cutaneous mani estations, shown in Fig. 5-75, are petechiae and purpura. T e “blueberry mu n” spots are an indication o extramedullary hematopoiesis.

Congenital rubella In utero in ection by the rubella virus during the rst 20 weeks o gestation may result in severe multisystem involvement and a variety o developmental de ects. Illustrated in Fig. 5-76 are the dark-blue-to-purple “blueberry mu n” lesions in the severely af ected neonate. T ey are a sign o extramedullary hematopoiesis. T rombocytopenic purpura is an additional cutaneous mani estation. Congenital cataracts, dea ness, and cardiac mal ormations orm a classic triad in in ants with the severe intrauterine in ection.

Fig ure 5-77

Fig ure 5-78

Rubella T is viral disease af ects both children and young adults. A mild prodrome consists o headache, coryza, and low-grade ever. T e rash itsel varies enormously in its course and duration. Most typically, it begins on the ace and neck and spreads to the trunk and extremities, as pictured in Fig. 5-77, over 1 or 2 days. T e lesions are small pink macules and maculopapules, which rapidly coalesce and then ade. T e rash is so evanescent that it may begin to disappear on the ace be ore developing on the trunk and extremities.

Petechiae on the so palate, termed Forchheimer sign, are present in some patients and are illustrated in Fig. 5-78. Systemic illness is generally mild. Enlargement o suboccipital and posterior auricular lymph nodes is a characteristic physical nding. T e most serious aspect o rubella is the severe developmental delay that may result rom intrauterine in ection during the rst trimester. Routine childhood immunization has resulted in ewer epidemics and there ore in a lower incidence o viral exposure to pregnant women.

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Fig ure 5-79

Fig ure 5-80

Measles Rubeola, or measles, is a systemic illness caused by an RNA paramyxovirus. T e incidence o this previously common childhood disease has decreased markedly since the advent o an ef ective vaccine, but measles has by no means been eradicated. A er an incubation period o 1 to 2 weeks, the in ected child develops ever, conjunctivitis, photophobia, and a distinctive brassy or barking cough. Frequently, the ever and constitutional symptoms are severe.

A day or two a er the onset o these constitutional signs and symptoms, the enanthem (Koplik spots) appears as bright-red puncta with central blue-white ecks on the buccal mucosa opposite the second molars (Fig. 5-79). T e characteristic rash is maculopapular and erythematous. It begins on the orehead and behind the ears and spreads to the ace (Fig. 5-80), neck, trunk, and extremities. T e rash disappears in the same sequence, sometimes leaving areas o ne desquamation. T e duration o the entire illness is usually 5 to 7 days.

Fig ure 5-81

Fig ure 5-82

Measles Figure 5-81 shows the “measly” appearance o a child who clearly eels miserable. T e disease is brie and sel -limited or most children, and the treatment is entirely supportive. T e incidence o complications is higher, however, than in other childhood exanthems. Otitis media is the most common. Serious complications include bronchopneumonia and encephalitis, which may cause permanent neurologic damage. Subacute sclerosing panencephalitis is a late sequela o measles.

Atypical measles T is can occur in individuals who were given the old killed-virus vaccine (no longer available) or were given a live measles vaccine that was inactivated by improper storage. Atypical measles is characterized by high ever and severe respiratory symptoms. T e accompanying rash is similar to that o Rocky Mountain spotted ever (Figs. 4-34 and 4-35). T e exanthem, which typically begins on the hands and eet, may be macular, vesicular, or purpuric.

65

Se ction 5 . Viral Dis e as e s

Fig ure 5-83

Fig ure 5-84

Erythema in ectiosum ( f h disease) Erythema in ectiosum is a mild childhood disease that is caused by human parvovirus B19. T is condition develops a er a mean incubation period o 14 days. T ere are ew i any prodromal symptoms. T e rash evolves in three clinical stages. T e rst stage is characterized by the abrupt appearance o a bright-red malar blush.

T e appearance is so startling that it has been given the suggestive description o “slapped cheeks” (Figs. 5-83 and 5-84). During the second stage, the acial rash begins to ade, and a maculopapular, urticarial, or morbili orm exanthem develops on the extremities and trunk. Pruritus may be present.

Fig ure 5-85

Fig ure 5-86

Erythema in ectiosum ( f h disease) As portions o this rash ade over a period o several days, a reticular pattern emerges, mainly on the extremities, which gives the arms and legs a marbled appearance (Fig. 5-85 and 5-86). T is reticular eruption, which is the third stage o the disease, may last or only 1 week, or it may last continuously or by relapses or as long as 8 weeks. Exercise and variations in temperature may make the rash appear more or less prominent. No treatment is required.

Human parvovirus B19 is also the cause o transient aplastic crisis in children with hemoglobinopathies, and o hydrops etalis in the children o women exposed to the virus during pregnancy. In addition, adults who are exposed to children with h disease may acquire human parvovirus in ection, and are likely to develop severe arthralgias as part o their illness. It is o note that children with h disease are contagious only be ore the occurrence o the rash.

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Fig ure 5-87

Fig ure 5-88

Papular purpuric sock and glove syndrome In its most characteristic orm, patients develop a papular and purpuric eruption on the hands and eet, with sharp demarcation at the wrists and ankles. Other areas o involvement may include the ace, buttocks, and inguinal creases.

T is syndrome was originally described in conjunction with parvovirus B19 in ection, but it has also been attributed to measles virus, cytomegalovirus, coxsackie virus B6, human herpesvirus 6, and hepatitis B. Systemic symptoms are rare and the condition usually resolves in 1 to 2 weeks.

SECTION

6 Super cial Fungal In ections

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Fig ure 6-1

Fig ure 6-2

Tinea corporis Super cial ungal in ections o the skin are among the most common o all pediatric dermatoses. T ese are illustrations o more super cial ungal in ections o hairless skin. T e annular lesions in Figs. 6-1 and 6-2 resulted rom in ection with Trichophyton tonsurans.

In the cases illustrated, clinical diagnosis o a super cial ungal in ection is reasonably certain, and one may guess that the causative ungus is a Microsporum or Trichophyton. A potassium hydroxide preparation o a scale obtained rom the edge o a lesion will identi y the hyphae.

Fig ure 6-3

Fig ure 6-4

Tinea corporis In some cases, tinea corporis presents with concentric rings (Fig. 6-3). De nitive diagnosis depends on mycologic culture o the scale rom a lesion. In cases o candidiasis, tinea imbricata and avus, the causative organism can requently and con dently be guessed correctly. In general, one may say that Microsporum canis, Microsporum audouinii, T tonsurans, and T schoenleinii can in ect scalp and hairless skin, and Trichophyton rubrum and Candida albicans can in ect hairless skin and nails.

Tinea corporis-vesicular On rare occasions, a marked in ammatory response to the presence o a dermatophyte leads to a vesicular or bullous eruption. T e clinical presentation seen in Fig. 6-4 must be di erentiated rom acute contact dermatitis, bullous impetigo, or an autoimmune blistering disease such as linear IgA dermatosis.

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Se ction 6 . Supe rf cial Fungal In e ctions

Fig ure 6-5

Fig ure 6-6

Tinea corporis T e numerous scaly rings illustrated in Figs. 6-5 and 6-6 are due to M canis. T e majority o cases result rom exposure to in ected cats, many o which have no symptoms o ringworm.

Kittens appear to have a higher requency o in ection than adult cats, and shedding o the ungus occurs more during the winter months. In addition, cats that live both outdoors and indoors appear to be more commonly in ected with M canis than do indoor cats.

Fig ure 6-7

Fig ure 6-8

Tinea corporis ( aciei) In ection at the sites shown in Fig. 6-7 may also be termed tinea aciei. Note the ring shape o the active periphery o the lesions in this patient.

Facial lesions may be due to T tonsurans, T rubrum, or T mentagrophytes. Cutaneous in ection with zoophilic species, such as M canis, may also occur on the ace. As noted in Fig. 6-8, this usually results rom close contact with a household pet.

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Fig ure 6-9

Fig ure 6-10

Tinea corporis ( aciei) opically applied anti ungal agents, such as the azole and allylamine agents, are e ective therapies. T ere may be temporary hyperpigmentation or hypopigmentation a er success ul treatment, but even severe lesions such as these resolve without scarring.

In some patients, the occurrence o ungal in ection in locations such as the ear may make it dif cult to appreciate the ring morphology o the lesion, and thus lead to misdiagnosis as atopic or contact dermatitis. In that situation, the application o topical steroid creams will make the in ection worse.

Fig ure 6-11

Fig ure 6-12

Neonatal tinea inea corporis can occur during the rst weeks or months o li e, and is sometimes misdiagnosed because the primary care provider has not considered that possibility.

T is in ant developed typical arcuate lesions on the ace. T e child’s caretaker had a ungal in ection o the hand.

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Se ction 6 . Supe rf cial Fungal In e ctions

Fig ure 6-13

Fig ure 6-14

Tinea incognito T is Latin term or “tinea unrecognized” re ers to lesions o tinea corporis that are made worse by the application o topical corticosteroids. T e areas o involvement may become unusually large and develop multiple concentric rings.

In this case o tinea incognito, the patient applied a topical corticosteroid, previously prescribed or atopic dermatitis, to an undiagnosed ringworm in ection on the ace. T e papules and nodules develop when ungal elements penetrate the hair ollicle and cause a deeper in ection. T is orm o in ection, termed Majocchi granuloma, may also occur on the legs o adolescent girls, where it is spread by shaving.

Fig ure 6-15

Fig ure 6-16

Tinea capitis Fungal in ections o the scalp are extremely common in children. T e diagnosis o tinea capitis should be entertained in any child in whom patches o incomplete alopecia, crusting, or scaling are ound in the scalp. In previous decades, M canis and M audouinii were the most common pathogenic ungi in ecting the scalp. T e latter requently causes a discrete grayish patch o hair loss, as shown in Fig. 6-16.

In most parts o the United States and in many other parts o the world, T tonsurans is now the predominant organism causing tinea capitis. In the United States, this orm o tinea capitis is seen almost exclusively in A rican-American children. In some children, this dermatophyte causes discrete and dramatic areas o hair loss studded by the stubs o broken hair, so-called blackdot ringworm (Fig. 6-18).

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Fig ure 6-17

Fig ure 6-18

Tinea capitis Because T tonsurans does not uoresce, the Wood’s light is no longer o use in most cases. However, diagnosis can be con rmed by the use o either potassium hydroxide preparation or ungal culture. Pictured here is a child with signi cant scaling and hair loss due to in ection with T tonsurans. T ere is also a small circular lesion on the acial skin. Similar lesions may develop on the back and chest and provide a clue to diagnosis.

In some cases, there are only small and unimpressive patches o “seborrheic” scale with minimal hair loss or groups o small pustules. Attempts to treat tinea capitis with topical anti ungal agents alone are doomed to ailure. Oral griseo ulvin is the most e ective orm o therapy. T e use o selenium sul de shampoo may be e ective in preventing spread to classmates and siblings.

Fig ure 6-19

Fig ure 6-20

Tinea capitis Figure 6-19 shows an exuberant in ammatory response to T tonsurans. T e lesions have spread onto the neck, but the primary source o in ection is in the hair ollicles in the scalp, and systemic therapy is required.

Kerion In some children, an exuberant in ammatory response to the in ecting dermatophyte may occur. T is boggy and tender mass is termed a kerion, a word that in Greek means a honeycomb, honey, or beeswax and is intended to describe the clinical appearance. Children with a kerion may also develop localized lymphadenopathy and ever. Although bacterial superin ection may sometimes occur, misdiagnosis o a kerion as a bacterial in ection o the scalp is an all too common pit all. T e proper treatment is oral griseo ulvin along with, in some cases, systemic therapy or the bacterial component.

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Se ction 6 . Supe rf cial Fungal In e ctions

Fig ure 6-21

Fig ure 6-22

Kerion Children who develop a severe in ammatory response to in ection with T Tonsurans may develop signi cant and prolonged hair loss a er the condition has been adequately treated. Even in cases that are as severe as this (Fig. 6-21), it is most common or the hair to return to normal.

Id reaction to tinea Lymphadenopathy is a common occurrence in children with tinea capitis. Be ore treatment or shortly a er starting griseo ulvin or tinea capitis, some patients also develop a pruritic eruption that is a hypersensitivity reaction. T ese patients develop numerous ne papules, concentrated on the ace, neck, trunk, and upper extremities. T ere is no need to discontinue the griseo ulvin, or the eruption resolves in 1 to 2 weeks. Relie can be obtained with the application o lowpotency topical corticosteroids. It is important to di erentiate this rom a true drug eruption.

Fig ure 6-23

Fig ure 6-24

Tinea corporis secondary to scalp in ection with T tonsurans Children with tinea capitis requently develop small round scaly lesions on the ace, upper arms, and neck, as pictured in Fig. 6-23. T ese can be treated with a topical anti ungal medication, but the underlying scalp in ection must be treated with a systemic medication. Frequent recurrences o lesions o this type provide an important clue to in ection o the scalp.

It is not unusual or young amily members o children with tinea capitis to also develop the same condition. Adult caregivers are likely to develop small round lesions without scalp involvement, and this can also be a clue to the diagnosis in the child. In Fig. 6-24, the patient’s mother developed such lesions on the inner aspects o her arms, probably rom the contact that occurred while combing her daughter’s hair.

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Fig ure 6-25

Fig ure 6-26

Tinea cruris T e groin is a common site o acute and then enduring super cial ungal in ection (Figs. 6-25 and 6-26). Epidermophyton f occosum, T rubrum, and C albicans are the most common in ecting ungi. In in ants, C albicans is the usual pathogen; in older children and adults, all are common.

Intertriginous spaces, such as groin, axillae, and digital webs, are particularly susceptible to ungal in ection because the pH o these areas is less acidic than elsewhere, temperature is higher, and humidity is greater.

Fig ure 6-27

Fig ure 6-28

Tinea pedis Super cial ungal in ection o the eet is somewhat unique because o the location. Between the toes (most commonly the ourth and h), the condition appears as erythema, maceration, and scaling. It is attended by itching or vague discom ort. On the sole and the lateral aspects o the eet, scattered pustules and vesicles with surrounding erythema and edema may occur. More commonly, there is persistent dry scale in a “moccasin” distribution with minimal in ammation.

In some cases, as shown in Fig. 6-28, this may become secondarily in ected. T e ungi that are commonly ound are E f occosum, T mentagrophytes, T rubrum, and C albicans. Fungal in ections o the eet, although rare in in ants and young children, must be considered when presented with erythema and scaling o the eet. More likely diagnoses with this presentation are atopic dermatitis, juvenile plantar dermatitis, and contact dermatitis.

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Se ction 6 . Supe rf cial Fungal In e ctions

Fig ure 6-29

Fig ure 6-30

Tinea pedis Figure 6-29 shows a typical “moccasin distribution” with scaling along the side o the eet. Sweating, riction, and debris promote ungal in ection. During early adolescence, there is a marked increase in the incidence o tinea pedis.

Occasionally, intensely pruritic vesicles along the side o the oot are a key to diagnosis. For most patients, the use o topical anti ungal therapy, along with the wearing o sandals and light cotton socks, is an adequate therapy.

Fig ure 6-31

Fig ure 6-32

Onychomycosis inea unguium ( ungal in ection o the nails) is somewhat uncommon during childhood. A er puberty, its requency increases with age. Usually, onychomycosis is associated with tinea pedis. Fungal culture o the nails is sometimes dif cult but extremely important in con rming the diagnosis. Onychomycosis is the most dif cult o the super cial ungal in ections to treat because the nail plate is not easily penetrated by topically applied agents. However, lacquer medications containing anti ungal agents are sometimes e ective.

Systemic anti ungal agents such as itraconazole and terbina ne are e ective in the treatment o onychomycosis, but laboratory monitoring may be necessary during treatment. Figures 6-31 and 6-32 show in ection with dermatophytes. Note the thickening and discoloration o nail plates in both photos. In ection by dermatophytes usually proceeds in a distal-to-proximal direction. In ection o nails by C albicans is di erent in that it is more acute ( requently purulent) and tends rst to involve the lateral and proximal nail olds.

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Fig ure 6-33

Fig ure 6-34

White superf cial onychomycosis T is relatively rare orm o onychomycosis usually a ects the toenails. It occurs when ungi directly invade the super cial part o the nail plate and orm opaque “white islands” on the plate. As this worsens, the nails become so t and rough. he most common cause is T mentagrophytes.

Tinea manuum Super cial ungal in ection o the palms usually presents with erythema and whitish scale, predominantly in the palmar creases. It is almost always associated with tinea pedis. Generally tinea manuum involves one hand, but when both hands are involved, it is not symmetrical. T e causative organism is usually T rubrum. T e condition may be treated with either topical imidazole creams or with oral anti ungals. On the dorsa o the hands, the in ecting ungi are more likely to be those that cause tinea corporis elsewhere.

Fig ure 6-35

Fig ure 6-36

Tinea versicolor T is title designates a super cial ungal in ection (tinea) that changes color (versicolor). T e causative organism was originally called M ur ur and is now more commonly called Pityrosporum orbiculare. inea versicolor typically causes numerous patchy scaling macules on the upper chest and back, proximal arms, and neck.

T e lesions may be hypopigmented, as shown in Fig. 6-36, or brown-orange, depending on the skin color o the patient and the degree o recent sun exposure. T e organism is believed to prevent either the ormation o melanin or the trans er o melanosomes into keratinocytes. T e ormation o azelaic acid is another suggested mechanism or the resultant hypopigmentation.

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Se ction 6 . Supe rf cial Fungal In e ctions

Fig ure 6-37

Fig ure 6-38

Tinea versicolor Although it is most common on the back and chest, involvement o the neck and ace are o en seen, especially in hot and humid environments.

inea versicolor is usually asymptomatic but may itch slightly. T e organism cannot be cultured, but diagnosis is aided by the orange or brown glow o lesional skin under a Wood’s light and by the “spaghetti and meatballs” appearance o clustered hyphae and spores on potassium hydroxide preparation.

Fig ure 6-39

Fig ure 6-40

Tinea versicolor Although tinea versicolor usually makes its appearance a er puberty, it can develop in childhood and acial lesions are even occasionally seen in breast- ed in ants.

T ere are several approaches to the treatment o tinea versicolor. Small areas o involvement can be eradicated by the use o an imidazole cream. In patients with more extensive disease, the best approach is the use o a selenium sul de or sodium thiosul ate lotion. Patients should be warned that treatment o tinea versicolor does not diminish the very high rate o recurrence and that the return to normal skin color may be delayed or weeks to months a er the completion o therapy.

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Fig ure 6-41

Fig ure 6-42

Tinea nigra T is is an in ection o the stratum corneum, commonly on a palm, that is caused by the dematiaceous ungus Exophiala (Cladosporium werneckii). It occurs most commonly in tropical regions. T e clinical appearance is a tan-to-black discoloration with sharp borders that enlarge slowly.

inea nigra can be mistaken or a melanocytic nevus, melanoma, or simple arti act. T e correct diagnosis can be con rmed by potassium hydroxide examination or ungal culture. reatment consists o a keratolytic lotion or topical imidazole cream.

Fig ure 6-43

Fig ure 6-44

Tinea imbricata inea imbricata is a super cial ungal in ection o the glabrous skin that is seen in a number o tropical countries. T e causative organism is T concentricum. Brown papules evolve into annular plaques. As the in ection continues, many concentric ring lesions develop, which resemble the patterning o a tortoise shell. Oral anti ungals are e ective therapy but relapses occur.

Favus T is chronic orm o tinea capitis is caused by T schoenleinii. It occurs in parts o Europe, A rica, and South America as well as in some rural areas o the United States and Canada. Pictured here are the typical scaly patches seen in avus. T e yellowish cup-like crusts, called scutula, are a distinctive part o this disease. T e coalescent crusts orm a plaque that emits a characteristic mousy odor. T is orm o ungal in ection o the scalp may persist into adult li e.

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Se ction 6 . Supe rf cial Fungal In e ctions

Fig ure 6-45

Fig ure 6-46

Congenital cutaneous candidiasis T e presence o a generalized candidal dermatitis at birth is the result o intrauterine in ection.

ypically, erythematous papulovesicles and macules evolve into pustules and areas o super cial desquamation over a period o 6 to 8 days.

Fig ure 6-47

Fig ure 6-48

Congenital cutaneous candidiasis I congenital candidiasis involves only the skin, the disease ollows a benign course. In ants with low birth weight may develop disseminated in ection or respiratory distress and require systemic therapy.

T e characteristic lesions requently involve the palms and soles and may be accompanied by paronychias and true nail involvement. In contrast to neonatal candidiasis (acquired during passage through an in ected birth canal), the diaper area and oral mucosa tend to be spared.

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Fig ure 6-49

Fig ure 6-50

Candidiasis (moniliasis) Cutaneous in ection with C albicans tends to occur in areas that are chronically moist, warm, and macerated. For this reason, Candida is among the most common causes o diaper dermatitis. Distinctive eatures sometimes include con uent, glistening, bee y-red erythema, and numerous small satellite lesions.

More extensive involvement is seen in Fig. 6-50; note the peripheral scaling and again the satellite lesions. On occasion, these peripheral pustules and papules will involve the entire abdomen and back. Diagnosis o cutaneous candidiasis can be con rmed by potassium hydroxide preparation or by ungal culture. reatment consists o the use o imidazole creams, such as ketoconazole, or nystatin.

Fig ure 6-51

Fig ure 6-52

Chronic mucocutaneous candidiasis T e immunologic inability to handle C albicans in ection results in recurrent and severe in ection o the mucous membranes, skin, and nails. A ected children have -cell de ciencies due to a wide variety o underlying causes but mani est their mucocutaneous disease in similar ashion. Chronic mucocutaneous candidiasis may, in some patients, lead to granuloma ormation in the areas o in ection. T e ace is a common site or the ormation o these indurated and hyperkeratotic plaques.

Figure 6-52 illustrates typical nail involvement in chronic mucocutaneous candidiasis. Patients develop swelling and tenderness o the proximal and lateral nail olds. Eventually, the nails become brittle and discolored and may be destroyed by the process. Chronic mucocutaneous candidiasis is sometimes part o an autoimmune polyglandular syndrome and such patients may su er rom a wide variety o endocrine abnormalities, along with vitiligo and alopecia areata.

SECTION

7 Deep Fungal In ections

82

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Fig ure 7-1

Chromoblastomycosis T e granulomatous process shown in Fig. 7-1, which is variegated in color, is o 12 years' duration. It started as a small nodule, developed slowly into a verrucous mass, and acquired satellite extensions. T e condition, so reminiscent o a tuberculous process, is a deep ungal in ection caused by the species o Phialophora, Fonsecaea, and Cladosporium, which are indigenous to parts o South America and other regions with warm climates. Lesions that are too large or surgical excision are treated with combinations o systemic ucytosine, amphotericin B, and ketoconazole.

Fig ure 7-2

Fig ure 7-3

Coccidioidomycosis T is disease, caused by the dimorphic ungus Coccidioides immitis, is endemic to the southwestern United States and parts o Central and South America. Most individuals in those areas develop the disease as an inconsequential upper-respiratory in ection.

Severe disseminated disease can ollow pulmonary in ection and may involve the skin. In Figs. 7-2 and 7-3, the lesions are typically abscesses, nodules, or verrucous and in ammatory plaques. Primary in ection o the skin, which is rare, is accompanied by regional lymphadenopathy. Amphotericin B, with or without itraconazole or uconazole, is the customary treatment.

83

Se ction 7 . De e p Fungal Infe ctions

Fig ure 7-4

Fig ure 7-5

Sporotrichosis Cutaneous in ection with Sporothrix schenckii is a disease with worldwide distribution. T e majority o cases are seen in Central and South America but outbreaks occur in the United States. T e disease a ects both children and adults and occurs when the causative ungus, in either contaminated soil or plant materials, contacts traumatized skin.

T e thorn o a rose bush may provide both the organism and a site o entry, and there have been outbreaks among children playing among bales o hay. Pictured here is the most common orm o sporotrichosis, the lymphocutaneous type. It causes an ulcerated lesion at the site o inoculation (Fig. 7-4) and a string o nodules or ulcerations along the lines o lymphatic drainage (Fig. 7-5).

Fig ure 7-6

Fig ure 7-7

Sporotrichosis T is is an example o xed cutaneous sporotrichosis on the ace. Lesions o this type are usually attributed to exposure during outdoor play. Speci cally, acquisition o S schenckii has been attributed to playing in bales o hay or in sphagnum moss. ransmission rom a cat has also been described.

T ese tender secondary lesions may become chronic and extend into subcutaneous tissues. Very rarely, and especially in the immunocompromised host, disseminated or systemic sporotrichosis ollows cutaneous in ection. Diagnosis o sporotrichosis can be con rmed by culture o exudate or tissue rom the skin lesions on Sabouraud agar.

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Fig ure 7-8

Fig ure 7-9

Mycetoma Also known as Madura oot, this is a chronic granulomatous in ection which results in swelling, discharge, and an exudate that contains characteristic grains. It is most common in India and A rica, and is also seen in Brazil, Mexico, and Venezuela.

Mycetoma caused by microaerophilic actinomycetes (such as Nocardia and Streptomyces) is re erred to as actinomycetoma. Mycetoma that is caused by true ungi (Fusarium, Exophiala, Madurella, and others) is called eumycetoma. T e choice o treatment depends on the cultured organism, and lesions of en resolve with scarring, as shown in Fig. 7-9.

SECTION

8 Bites and In estations

86

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Fig ure 8-1

Fig ure 8-2

Insect “bites” Many e ects o metazoal parasitism are attributed to bites. Some metazoa (insects in a loose sense) do indeed bite, and others sting, but what we requently designate as insect bite is attachment or eeding. T e result o such attachment looks like a bite and is sooner or later attended by pain, itching, or stinging. rue bites and stings, however, are instantly pain ul; many have immediate or late, more bale ul e ects; and most are generally in icted in sel -de ense or seemingly wanton o ense, not or eeding.

Attachment or eeding is parasitism that may be silent or a while and then variably symptomatic. In a given region, common indigenous metazoa that cause cutaneous e ects by a bite, sting, or attachment or eeding may be recognized or guessed rom signs and symptoms. T ese two illustrations are representative. Figure 8-1 may be guessed with reasonable correctness to be mosquito “bites,” and Fig. 8-2, clustered on the lower leg and ankle, to be ea bites. Because di erent amily members may have di erent degrees o sensitivity, it is possible that only one or several in the household will develop these lesions, and others will be spared.

Fig ure 8-3

Fig ure 8-4

Insect “bites” T e location o bites gives important clues to the causative insects. T ose pictured in Figs. 8-3 and 8-4 are unlikely to be rom mosquitoes (which tend to bite on exposed areas o skin) or eas (which tend to bite the ankles and lower legs). A variety o crawling insects and mites can cause lesions o this sort, including chiggers.

Chigger bites are caused by trombiculid mites. T ese mites live in tall grasses and weeds and are most active in summer and all. T ey attach to the skin as one walks by and brushes up against the vegetation. Lesions tend to occur in areas that are warmer, moister, or covered by tight clothing. T e intense itching may last or several days.

87

Se ction 8 . Bite s and Infe s tations

Fig ure 8-5

Fig ure 8-6

Insect “bites” Certain insects creep under clothes and bite when they reach a point o restriction such as a sock or belt. Figure 8-5 shows bite marks and vast edema on the penis. T e assaulting insect may have been an ordinary one that merely took advantage o a child le undressed and unguarded.

When a subject has been bitten, stung, or ed upon, the consequent lesion may be typical at the time and in its course to resolution or may be modi ed by scratching, secondary in ection, or idiosyncratic host response. In the case o the mosquito, the lesions may be one, ew, or many, depending on the insensitivity, oolhardiness, or de enselessness o the subject assailed.

Fig ure 8-7

Fig ure 8-8

Insect “bites” T e vesicular lesions in Fig. 8-6 and the bullous lesions in Fig. 8-7 are evidence o a strong hypersensitivity reaction to the o ending insect.

Dermatitis caused by the common carpet beetle T e common carpet beetle is not common in modern well-sanitized homes, and even where it is abundant, e ects rom it are not common. Nevertheless, on occasion, the crawl o the insect on an unwary individual leaves its marks as wheals, papulovesicles, or bullae. Figure 8-8 shows just such an event in the orm o large, accid blisters. T e three lesions in a line record the walk o a creature and the deposition o its irritant principle.

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Fig ure 8-9

Spider bites Spiders, timid creatures or all their erce looks, would rather entertain insects in their parlors than attack humans. T e tarantula is more dangerous in able than in act. wo spiders can deliver pain ul bites and serious veneration i rightened or cornered: the black widow spider (Latrodectus mactans) and the brown recluse spider (Loxosceles reclusa). T e bite o the latter is illustrated in Fig. 8-9. It is a hemorrhagic bleb; in time it will become a necrotic ulcer. T e patient will be severely sickened.

Fig ure 8-10

Fig ure 8-11

Papular urticaria T is is a pruritic dermatosis that is causally related to bites by mosquitoes, eas, or other insects. Children with this common disorder tend to have recurrent episodes during the spring and summer months. T e lesions consist o rm erythematous papules, sometimes with surrounding wheals. T ey avor exposed areas, especially the anterior lower extremities and lower arms. T e individual papules are o en excoriated and are sometimes impetiginized.

T e lesions tend to last or several days to weeks but will persist longer i chronically scratched or rubbed. Papular urticaria is more common among children with atopic diathesis and represents a hypersensitivity reaction to the assaulting arthropod. reatment consists o lotions, topical steroids, and antihistamines. T e elimination o the o ending insect rom the household environment is help ul but will not prevent encounters during outdoor play.

89

Se ction 8 . Bite s and Infe s tations

Fig ure 8-12

Fig ure 8-13

icks Figure 8-12 shows an engorged deer tick attached to its human host. icks o the Ixodes species are vectors o Lyme disease as well as hemorrhagic evers and viral encephalitis. Most tick bites are not pain ul and there ore may go unnoticed by patients or variable periods o time. A tick bite may appear as a red papule or may progress to erythema with local swelling, blistering, or ecchymosis. Chronic tick bite granulomas can develop and last or months to years.

ick “bite” ick in estation occurs when the emale so or hard tick inserts her proboscis into the skin in order to withdraw blood. T e site o attachment may develop into an erythematous nodule, and persistent pruritus may result i tick parts are le within the skin. Although most tick bites are insigni cant, these insects are the vectors o tick bite ever, Rocky Mountain spotted ever (Figs. 4-34 and 4-35) and erythema chronicum migrans (Lyme disease) (Figs. 4-8 to 4-11).

Fig ure 8-14

ick bite granuloma Sometimes at the site o a tick bite, a persistent rm papulonodular lesion may develop. A common site or this reaction to develop is in the scalp. T is area may be very pruritic and with excoriation may result in secondary in ection.

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Fig ure 8-15

Fig ure 8-16

Scabies In ection o the skin by the human scabies mite (Sarcoptes scabiei var. hominis) is an extremely common skin disease o childhood. Figure 8-15 shows a child with multiple intensely pruritic lesions on the trunk. Examination o adult amily members or lesions in the nger webs, waist line, and wrists provide a clue to the diagnosis in the child.

Figure 8-16 shows another severe case o scabies. T e in ant with scabies illustrated here has numerous excoriated papules and a di use eczematous dermatitis. In in ants, scabies requently involves the entire cutaneous sur ace including the ace and scalp. Young in ants without apparent pruritus may mani est extreme irritability.

Fig ure 8-17

Fig ure 8-18

Scabies In ammatory nodular lesions involving the axillae and the diaper area are particularly typical o scabies in the very young child. T ese lesions may coexist with burrows, papules, vesicles, pustules, and areas o crusting.

T e nodules may persist or quite some time a er the in estation is treated with a medication such as topical permethrin, and their presence does not re ect a persistent in estation. I no new lesions are developing, the persistent nodules can be treated with topical corticosteroids.

91

Se ction 8 . Bite s and Infe s tations

Fig ure 8-19

Fig ure 8-20

Scabies T e location o primary lesions is o en help ul in establishing the diagnosis o scabies. In young children, the palms and soles are sites o predilection. In older children and adolescents, the most common sites or the intensely pruritic lesions are the anterior axillary lines, the inner aspect o the upper arm, the areolae, the penis, the wrists and interdigital webs, and the ankles (Figs. 8-19 to 8-21).

T e pre erred treatment o scabies is a single overnight application o a 5 percent permethrin cream, repeated 1 week later. T e application o any scabicide must be accompanied by a thorough laundering o all recently worn clothing and o bed linen, which may still contain mites or eggs. All close contacts should be treated.

Fig ure 8-21

Fig ure 8-22

Scabies Figure 8-21 illustrates the presence o numerous papulovesicular lesions on the sides o the eet in a child with scabies.

Figure 8-22 shows a scabies mite. T e mite has an ovoid body with our pairs o short legs. Eggs and eces (scybala) are deposited in the burrows by the emale mite, and it is common to nd all or some o these elements in a scraping o a burrow.

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Fig ure 8-23

Fig ure 8-24

Scabies Children and adolescents o en develop lesions on the dorsum o the hands and between the ngers. T is is an area where burrows are commonly seen.

Figure 8-24 illustrates numerous burrows on the dorsal ngers. T e black arrows are only pointing to a ew o the burrows that are present.

Fig ure 8-25

Fig ure 8-26

Scabies Figure 8-25 illustrates burrows that are diagnostic o scabies. Burrows are represented by slightly raised white to lightbrown linear lesions. T e super cial part o the burrow has a scaly appearance, and at the distal end there may be a tiny black dot representing the mite, eggs, and/or ecal material (scybala) in a small vesicle.

Scabies T is is a photograph o what is called crusted or Norwegian scabies. Individuals with this orm o disease develop thick areas o heavy crusting. Favored locations or the hyperkeratotic plaques include the elbows, knees, scalp, and buttocks. Norwegian scabies occurs most commonly in patients with Down syndrome and among those who are debilitated or who su er rom an immune de ciency. In crusted scabies, the areas o involvement contain numerous mites, and there ore the disease is highly contagious.

93

Se ction 8 . Bite s and Infe s tations

Fig ure 8-27

Postscabetic acropustulosis In ants and children who are success ully treated or scabies may subsequently develop recurrent crops o intensely pruritic papules and pustules on the hands and eet. I persistent scabies in estation has been ruled out, this orm o id reaction should be treated with topical corticosteroids. T is persistent dermatitis appears to be most common in developing countries, and also in the context o international adoption.

Fig ure 8-28

Fig ure 8-29

Pediculosis capitis (head lice) T e head louse is a wingless six -legged insect that is the smallest o the three human lice. It is adapted to live in the hair o the scalp only and lives by eeding on blood. T e emale louse lays approximately our eggs per day and has a li e span o 2 to 4 weeks.

It uses a gluey substance to attach the egg cases or nits to the hair. Note the live louse in Fig. 8-28 with some nits in the scalp above the ear. Numerous nits are noted in the occipital scalp in Fig. 8-29.

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Fig ure 8-30

Fig ure 8-31

Pediculosis capitis (head lice) T e diagnosis o pediculosis capitis is suggested by the presence o itching in the posterior scalp or signs o redness, a papular eruption as noted in Fig. 8-30, or olliculitis at the nape o the neck. Note the numerous nits in the scalp above the papular neck eruption.

T e diagnosis o head lice is con rmed by the presence o nits, which, even when sparse, are easily recognized with handlens magni cation. T e nit can be recognized by examining an in ested hair under the microscope; a close-up is shown in Fig. 8-31.

Fig ure 8-32

Fig ure 8-33

Pediculosis corporis T e body louse belongs to the genus Pediculus; it is larger and broader than the head louse. While the head louse in ests the skin o the scalp, the body louse does not live on the body. It lives in the seams o undergarments and comes onto the body only to eed. Clinically, one sees marks o eeding and excoriations; the lice themselves can be ound in the clothes. reatment consists o disin estation o clothing and bedding and the application o a pediculicide to the patient.

Pediculosis pubis T e pubic louse (Phthirus pubis) is aptly called the crab louse. Examined under the microscope, this insect is broad like a crab and has legs that look like claws. On the skin, the louse looks like a brown eck and may be mistaken or a reckle. Nits are also easily seen grossly and better by hand-lens magni cation. Figure 8-33 is a good representation. In estation may also occur in the eyelashes and axillary hair.

95

Se ction 8 . Bite s and Infe s tations

Fig ure 8-34

Macula ceruleae T ese bluish macules are the result o the bite o Pthirus pubis, the pubic or crab louse. Most likely, the color results rom an anticoagulant that is contained in the saliva o the organism. In sexually active adolescents, pubic lice may probably be acquired rom an in ested partner. Young children may acquire this in estation rom innocent contact with an a ected adult, but the possibility o sexual abuse should also be considered.

Fig ure 8-35

Fig ure 8-36

Myiasis T e term or in estation with the larvae o ies is pronounced mī’yĕ-sĭs or mī-ī’ ĕ-sĭs. In this condition, ies deposit ertilized ova upon neglected wounds or under onycholytic nails. T e in estation is usually a sign o poor hygiene. Dermatobia hominis, Wohl ahrtia vigil, and several other species o bot ies and warble ies may oster their young in this way. Antibiotics may be needed to treat secondary in ection.

Figure 8-35 shows a cutaneous lesion but does not reveal the larvae, which can be better seen in movement in vivo by the naked eye. Figure 8-36 shows a larva clearly. In most cases, the larvae can be easily removed mechanically under local anesthesia.

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Fig ure 8-37

Fig ure 8-38

Caterpillar dermatitis T e hair o caterpillars and moths may produce pruritic or pain ul in ammatory skin lesions. T e puss caterpillar and the brown-tail moth are the most common o enders. T e toxin on the spines and hair o these insects causes an irritant dermatitis. T e resultant lesions, which are sometimes seen in linear array, may be papular or urticarial and are accompanied by itching or stinging. Systemic symptoms are rare but may occur. T e hair may be seen microscopically in skin scrapings. reatment is purely symptomatic.

Dermatitis caused by blister beetles T ere are several species o beetles (order Coleoptera) that secrete cantharidin or some other vesicant substance onto their bodies. T e crawl o the so-called blister beetles smears their irritant substances on the skin, causing the edema and blistering illustrated in Fig. 8-38. reatment with cool compresses and topical antibiotics to prevent superin ection is usually adequate.

Fig ure 8-39

Fig ure 8-40

Seabather’s eruption his eruption is an acute, pruritic dermatitis that occurs under covered areas a er one bathes in seawater. Pruritus begins shortly a er leaving the water, with the subsequent development o erythematous macules, papules, or urticarial lesions. Children may present with ever and chills. T ese intensely itchy lesions tend to resolve spontaneously over a period o 1 to 2 weeks. Recent reports have linked this eruption to the larvae o the phylum Cnidaria, which comprises jelly sh, corals, hydroids, and sea anemones.

Swimmer’s itch T is is an acute dermatitis produced by the cercarial orms o schistosomes and primarily occurs in uncovered areas o the body. When occurring on the ankles, this is sometimes called “clamdigger’s itch.” T e eruption may be acquired in resh water or saltwater. Like any intensely pruritic condition, excoriations and secondary in ection are complications. reatment consists o antipruritics and antibiotics when superin ection occurs.

97

Se ction 8 . Bite s and Infe s tations

Fig ure 8-41

Fig ure 8-42

T e sting o the Portuguese man-o -war One o the most pain ul e ects on skin is the consequence o attack by oceanic hydrozoans known as Portuguese men-o -war, which are amazing or their size, brilliant color, and power to induce whealing. T ey have a small oat that buoys them up and rom which hang long tentacles. T e wrap o these tentacles results in linear stripes, which look like whiplashes, caused not by the orce o their swing but rom deposition o urticariogenic and irritant substances.

T e ef ect o contact with a sea urchin Sea urchins are a gastronomic delight when prepared properly, but a cutaneous torture when stepped on unprepared. T e echinoids have spines that in some species are several inches long. Driven into skin when one steps or alls on or brushes against the creatures, the hard spines break and lodge in skin. Pain is inevitable, and secondary in ection is nearly inevitable i they are le in (Fig. 8-42). Nothing but tedious and meticulous extraction o every one o dozens, possibly scores, o spines is required or relie .

Fig ure 8-43

Fig ure 8-44

Larva migrans (creeping eruption) In estation o the skin with larvae o certain helminths, notably Ancylostoma braziliense, is common on the southern coast o the United States as well as in some other parts o the world. Helminths such as A braziliense in est the intestines o animals, particularly the dog, whose excreta contain larvae that have remarkable capacity to enter the unbroken skin.

Attaining the interior just below the stratum corneum, the immature orms move in a serpentine, erratic way, creating long channels. T e larvae are sometimes barely discernible at the ends o the channels in the direction o travel. T e eet, buttocks, arms, hands, and back are the common sites o lodgment and lesions. reatment consists o a topical application o thiabendazole.

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Fig ure 8-45

Fig ure 8-46

Larva migrans (creeping eruption) Figure 8-45 illustrates the typical linear lesions, along with vesicles and bullae.

T ese characteristic linear lesions, shown in Fig. 8-46, on the buttocks and labia occurred in an in ant who was unwittingly placed on a contaminated beach or lawn.

Fig ure 8-47

Fig ure 8-48

Onchocerciasis Onchocerca volvulus, such as Tunga penetrans, Dracunculus medinensis, Wuchereria bancrof i, and Loa loa, is limited to parts o the developing world. All these metazoa in est deeply and are dif cult to eradicate. T e larial elephantiasis caused by W bancrof i, the edema (Calabar swellings) around the eyes caused by the “A rican eye worm” (Loa loa), and the interdigital nodule, usually between toes, that harbors yards o the guinea worm (D medinensis) are endemic to varying regions o A rica, South America, and Asia.

Onchocerciasis is seen commonly in parts o Mexico. T e metazoan that causes this disease is o en ound in the scalp and around the eyes where in ection may have serious consequences. Figure 8-47 is a lumpy lesion on the scalp caused by the adult worm. Figure 8-48 shows less discernible lesions on the chest. T ese are caused by the organism in its micro larial phase.

SECTION

9 Atopic Dermatitis

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Fig ure 9-1

Fig ure 9-2

Atopic dermatitis T is condition is the most common o all pediatric dermatoses. For the majority o patients, the onset occurs during in ancy. T e classic acial appearance in this age group is illustrated in Figs. 9-1 through 9-4.

T ere are symmetrical patches o erythema, exudation, and scale involving the cheeks and chin. It is not unusual also to see widespread involvement o the trunk and extensor extremities during in ancy; the diaper area is most o en spared.

Fig ure 9-3

Fig ure 9-4

Atopic dermatitis Pruritus is a cardinal eature o atopic dermatitis and may be evidenced in the in ant by irritability, scratching, and rubbing against nearby objects.

Atopic dermatitis is an inherited disorder. Children with the disease most o en have a amily history o the atopic diathesis (atopic dermatitis, asthma, or allergic rhinitis) and may themselves mani est asthma or allergic respiratory disease.

101

Se ction 9 . Atopic De rm atitis

Fig ure 9-5

Fig ure 9-6

Atopic dermatitis Figure 9-5 shows a severe case o atopic dermatitis with early evidence o impetiginization.

Figure 9-6 shows a similar distribution in an older child. In this case, also, impetiginization may be contributing to the crusted appearance o the lesions.

Fig ure 9-7

Fig ure 9-8

Atopic dermatitis When the diagnosis o atopic dermatitis is in doubt, the search or associated clinical ndings is o en help ul. T e Dennie-Morgan line is a double old under the eye, which is seen in many children with atopic dermatitis.

Pictured here is another child with periocular involvement. Children who su er rom seasonal allergies with associated conjunctivitis and itching may have worsening o the erythema, scale, and licheni cation around the eyes during “allergy season”.

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Fig ure 9-9

Atopic Dermatitis Figure 9-9 shows another telltale sign: a issure at the junction o the pinna o the ear and the ace. Other associated indings related to atopic dermatitis include lichen spinulosus (Fig. 9-23), pityriasis alba (Figs. 9-24 and 9-25), ichthyosis vulgaris (Figs. 15-1 to 15-4), and keratosis pilaris (Figs. 15-63 and 15-64). Children with atopic dermatitis are also requently noted to have hyperlinear palms and soles. Keratoconjunctivitis and cataracts may occur in the child with atopic dermatitis.

Fig ure 9-10

Fig ure 9-11

Atopic dermatitis During childhood, the most common locations o atopic dermatitis are the antecubital and popliteal ossae and the posterior neck. Figures 9-10 and 9-11 show children in whom the involvement is more widespread. T e lesions show the characteristic erythema, oozing, and crusting o acute atopic dermatitis.

T e exact pathogenesis o atopic dermatitis remains a mystery. Patients with the disease seem to have altered autonomic unction and increased plasma histamine levels. Many have elevated levels o IgE, but some are completely normal in this respect. However, none o these observations has yet provided a basis or understanding the chronic and recurrent skin lesions o atopic dermatitis. An inherited abnormality in laggrin expression a ects barrier unction and seems to be associated with this disease in some children.

103

Se ction 9 . Atopic De rm atitis

Fig ure 9-12

Fig ure 9-13

Atopic dermatitis Figures 9-12 and 9-13 are illustrations o atopic dermatitis on the dorsum o the eet and the hand. T ese are among the common sites o involvement in somewhat older children. T e majority o children with atopic dermatitis experience improvement o their disease and many have normal skin as adults. However, the severe pruritus and the unpleasant appearance o the skin that accompany the exacerbations o this condition can be extremely troubling to the parent and child alike.

Food allergies sometimes play a role in severe eczema in in ants. T e role o diet in the treatment o atopic dermatitis in older children remains controversial. Despite evidence that some children with atopic dermatitis display a signi cant histamine response to some oods, it is air to say that the majority o children with the disease do not respond to modi cations o their diet. However, any oods that cause an obvious worsening o the condition should be avoided. Occasionally, ood allergy in the atopic child may even be mani ested by the rapid evolution o contact urticaria on the ace and hands.

Fig ure 9-14

Fig ure 9-15

Atopic dermatitis Figures 9-14 and 9-15 illustrate skin that is scaly and licheni ed. T e treatment o atopic dermatitis should include the establishment o a routine o skin care that maintains adequate hydration o the skin.

Parents should be encouraged to apply a lubricating ointment to the child’s skin several times a day. opical corticosteroid ointments are currently the mainstay o medical management. Many children do well with proper use o the milder, non uorinated ointments, but some require stronger preparations or brie periods o time.

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Fig ure 9-16

Fig ure 9-17

Atopic dermatitis Figure 9-16 shows atopic dermatitis involving one toe. Figure 9-17 shows scaling and licheni cation o the entire oot and ankle exure. T e dangers o local atrophy and stria ormation and o systemic absorption must be considered care ully with the use o the stronger topical corticosteroids. Important adjuncts to therapy include topical calcineurin inhibitors, oral antihistamines and, occasionally, a brie course o oral antibiotics to reduce skin colonization with Streptococcus and Staphylococcus.

Important adjuncts to therapy include topical calcineurin inhibitors (tacrolimus and pimecrolimus), oral antihistamines, and, occasionally, a brie course o oral antibiotics to reduce skin colonization with Streptococcus and Staphylococcus. Very rarely, children with extremely severe disease require systemic therapy with drugs such as methotrexate, cyclosporine and azathioprine.

Fig ure 9-18

Fig ure 9-19

Juvenile plantar dermatosis Figures 9-18 and 9-19 show erythema and ssuring on the weight-bearing sur ace o the oot. T is disorder, which tends to be worse in the winter months, is called juvenile plantar dermatosis. It is much more common in children with atopic dermatitis.

Juvenile plantar dermatitis, which has also been called wet-dry foot syndrome, is caused by excessive sweating o the eet in occlusive ootwear and rapid drying in a low-humidity environment. T e use o emollient ointments is extremely help ul.

105

Se ction 9 . Atopic De rm atitis

Fig ure 9-20

Fig ure 9-21

Frictional lichenoid dermatitis Figures 9-20 and 9-21 illustrate ne, erythematous, and esh-colored papules on the elbows. T is pruritic eruption, also termed as summer prurigo or summertime pityriasis, occurs most commonly but not exclusively in children with the atopic diathesis.

T e rash is seen most requently between the ages o 4 and 10 years and tends to recur seasonally in the late spring or early summer. Some children also have lesions on the knees and dorsa o the hands. Children with allergic contact dermatitis rom nickel may develop a very similar rash as an id reaction.

Fig ure 9-22

Fig ure 9-23

Atopic dermatitis with follicular accentuation In children with skin o color, it is common or atopic dermatitis to present as clusters o small papules located at each o the vellus hair ollicles. his orm o eczema is also pruritic, and treatment or this condition is the same as or other causes o eczema.

Lichen spinulosus T is condition consists o a small aggregation o keratotic papules at the openings o the hair ollicles. T e ollicular hyperkeratosis is so marked that the involved area has an appreciable spiny texture, but the areas do not itch (Fig. 9-23). T e typical circular or oval plaque is present in Fig. 9-23. Lichen spinulosus is probably most common among children with atopic dermatitis. Ef ective treatment may be achieved by the use o topical preparations containing keratolytics, such as small percentages o salicylic acid (2%-3%), urea (10%-20%), and alphahydroxy acids.

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Fig ure 9-24

Fig ure 9-25

Pityriasis alba T is is a condition that is characterized by blotchy areas o hypopigmentation with or without scale. T e lesions are usually asymptomatic. In older children, the cheeks are a avored location, but in in ants, the process may be more generalized (Figs. 9-24 and 9-25). Both gures illustrate how the borders o the lesions tend to ade into the surrounding normal skin. T is contrasts with vitiligo (Figs. 27-12 through 27-17), another common cause o pigment loss. In vitiligo, the areas o involvement tend to be a whiter white and are more sharply demarcated.

Pityriasis alba is more common among children with atopic dermatitis and may represent a orm o postin ammatory hypopigmentation. T ere may or may not be a preceding in ammatory dermatosis in the same area. It is important to reassure the parent and child that the loss o pigment is temporary and that complete restoration o normal skin color will occur. T e use o a mild topical steroid or topical tacrolimus or pimecrolimus may speed the process.

Fig ure 9-26

Fig ure 9-27

Pompholyx (dyshidrotic eczema) T is is a chronic, recurrent eruption o the hands and eet that is o en accompanied by severe pruritus. It is considerably more common among individuals with an atopic history or with a amily history o atopic disease. Figures 9-26 and 9-27 show a number o deep-seated vesicles and pustules on the palm and sole.

Small, rm vesicles along the sides o the ngers are a particularly common clinical sign. At times, bullae may occur. Patients who develop pompholyx o en tend to have hyperhidrosis o the hands and eet. Some observers note that episodes o the disease are brought on by stress. Management o this condition is best achieved by the use o topical corticosteroid creams or ointments.

SECTION

10 Allergic and Irritant Contact Dermatitis

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We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 10-1

Fig ure 10-2

Allergic contact dermatitis (Poison ivy) Contact dermatitis is a cell-mediated delayed hypersensitivity response to a variety o di erent antigens. Acute lesions are characterized by erythema, vesiculation, and oozing, whereas chronic areas o involvement may be dry and licheni ed.

T e diagnosis o allergic contact dermatitis (ACD) is a simple one when there is a clear history o exposure to an allergen or when the distribution o the lesions provides a strong clue. At other times, the identi cation o the causative agent can be very di cult.

Fig ure 10-3

Fig ure 10-4

Allergic contact dermatitis (Poison ivy) A number o di erent plants are capable o causing contact dermatitis. By ar, the most common are members o the genus Toxicodendron: poison ivy, oak, and sumac. Figures 10-3 and 10-4 are illustrations o contact dermatitis rom poison ivy, the most common single cause o contact dermatitis in childhood.

T e linear array o vesicles and bullae in Figures 10-1 and 10-2 re ects the pattern in which the resin was trans erred rom lea to skin. Figures 10-3 and 10-4 show severe acial involvement and a more di use reaction. Children who experience recurrent episodes o this phytodermatitis should be encouraged to learn to recognize the causative plants.

109

Se ction 1 0 . Alle rgic and Irritant Contact De rm atitis

Fig ure 10-5

Fig ure 10-6

Allergic contact dermatitis (wet wipes) Wet wipes containing the preservative methylisothiazolinone are a cause o ACD. T is orm o ACD can be misdiagnosed as psoriasis, eczema, or impetigo. Discontinuation o wet wipes results in a complete resolution o this dermatitis.

Allergic contact dermatitis (disposable diapers) Allergic reactions to the chemical components o a disposable diaper, including dye, may present as pictured in Fig. 10-6. “Lucky Luke” or “cowboy holster” dermatitis has the pattern o a cowboy’s gunbelt, with triangular-shaped erythema beneath the side bands o the diaper on the lateral buttocks, anks, and upper lateral thighs.

Fig ure 10-7

Fig ure 10-8

Allergic contact dermatitis (mango) Contact dermatitis to mango may present as a chronic rash on the lips and around the mouth. Mango is a member to the Sumac amily, and its sap contains the oil Urushiol. It is important to inquire about recent mango consumption when diagnosing an eruption like the one pictured in Fig. 10-7.

Allergic contact dermatitis (neomycin) T e child in Fig. 10-8 has an ACD resulting rom the repeated application o a preparation containing neomycin. T e development or worsening o an eczematous eruption af er the use o a topical medication, either prescribed or over-the-counter, should alert the physician to the possibility o a contact dermatitis (dermatitis medicamentosa). In addition to neomycin, common o enders are the stabilizer ethylenediamine and the paraben preservatives.

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Fig ure 10-9

Fig ure 10-10

Allergic contact dermatitis (shoes) T e allergic contact dermatitis Fig. 10-9 was caused by a sandal. Note how the eruption con orms, like a patch test, to the design o the thongs. Shoe dermatitis should be considered in any eczematous eruption on the dorsum o the oot in a child. T e agent responsible or the rash in this photograph was a tanning agent or dye. T e other common causes o shoe dermatitis are rubber and rubber accelerators, adhesives, and leather. I the allergen is correctly identi ed by patch testing, it is possible to select the ootwear that is tolerable.

Allergic contact dermatitis Figure 10-10 demonstrates the use o patch testing done or determining the cause o an ACD and illustrates a positive reaction in the orm o erythema at 48 hours in a person who has been sensitized to the o ending agent.

Fig ure 10-11

Fig ure 10-12

Nickel contact dermatitis T e development o an itchy eczematous eruption near the umbilicus is virtually pathognomonic or contact dermatitis to nickel. T e source is the small metal snap in the blue jeans or the metal belt buckle. T e simultaneous occurrence o an id reaction, sometimes with small lichenoid papules on the elbows and knees, is very common.

Lesions can be treated e ectively with topical corticosteroids, but the only cure results rom strict avoidance o nickel. T is is easier said than done. Parents must buy jeans without nickel snaps or sew in a small piece o abric to protect the underlying skin. Families should be reminded that wearing jeans with a nickel snap or just several hours out o the month would reactivate the entire process.

111

Se ction 1 0 . Alle rgic and Irritant Contact De rm atitis

Fig ure 10-13

Fig ure 10-14

Nickel contact dermatitis Children with contact dermatitis to nickel should also avoid metal jewelry and should be advised against ear piercing. In ants may present with skin lesions corresponding to the location o snaps on their pajamas or other garments.

Nickel contact dermatitis Allergy to nickel is one o the most common causes o contact dermatitis. Older children and adolescents may show reactions to watches, chains, belt buckles, or earrings. Figure 10-14 shows an example o contact dermatitis in the metal clasp o a bra. It is possible to test or the presence o nickel by using a spot test kit, which is a liquid containing 1% dimethylglyoxime and 10% ammonium hydroxide.

Fig ure 10-15

Fig ure 10-16

Allergic contact dermatitis (Reaction to temporary tattoo) Contact allergy to temporary tattoos has become an increasingly common phenomenon. In most cases, the tattoo material does not contain pure henna, but is a mixture o brown henna with paraphenylenediamine (PPD) called black henna. T e patient shown in Fig. 10-15 is allergic to PPD in the tattoo.

In act, the concentration o PPD in black henna is higher than that seen in commercial hair dyes. Af er resolution o the eczematous skin eruption, postin ammatory hyperpigmentation may persist or a considerable period o time.

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Fig ure 10-17

Fig ure 10-18

Irritant diaper dermatitis T e most common orm o diaper dermatitis is related to the combination o moisture and riction. T e result is illustrated in Fig. 10-17. Note that the areas o involvement are olds o skin that are in direct apposition to the diaper itsel ; the skin creases tend to be spared. T e bright erythema and shiny appearance o the involved skin are typical. Rashes o this type must be di erentiated rom candidiasis, seborrheic dermatitis, diaper contact dermatitis, and psoriasis.

Erosive diaper dermatitis (dermatitis of Jacquet) In ammation that results rom the precipitating actors o wetness, heat, riction, and debris in intertriginous places varies directly with the intensity and duration o those precipitating actors. T e in ammation may be merely a slight erythema, a tumid, bee y redness with serous exudation, or an area o ulceration. T e punched-out ulcers illustrated in Fig. 10-18 are typical o the erosive diaper dermatitis o Jacquet.

Fig ure 10-19

Fig ure 10-20

T is severe irritant dermatitis responds to the use o barrier creams, mild topical steroids, and requent diaper changes (Fig. 10-19). Evaluation or candidiasis or bacterial in ection should be considered in those who do not respond to treatment.

Pseudoverrucous papules and nodules T is unique diaper dermatitis was originally described in the skin surrounding urostomies, but can occur as a result o chronic diarrhea (sometimes rom Hirschsprung disease), encopresis, or urinary incontinence. T e at topped or round lesions are shiny, erythematous, and moist (Fig. 10-20). T ey resolve completely when the problem causing chronic irritation in the diaper area has been corrected.

113

Se ction 1 0 . Alle rgic and Irritant Contact De rm atitis

Fig ure 10-21

Fig ure 10-22

Lip licking dermatitis Irritant contact dermatitis is an eczematous eruption that results rom the application o an irritating substance to the skin. Examples would include strong acids or alkalis, harsh soaps, and bleaches. In these cases, cell–mediated allergy is not involved. T e irritant in Fig. 10-21 is saliva. T e child pictured here has developed a habit o licking his lips.

Some children do this as compulsively as others suck their thumbs or bite their nails. Notice how the design o the in ammatory lesions con orms to the extent to which the lips can be drawn into the mouth or licked by the tongue. In Fig. 10-22, postin ammatory hyperpigmentation is shown in a child who has recovered rom in ammation induced by chewing and blowing bubble gum.

Fig ure 10-23

Shin guard dermatitis T ere is controversy as to whether this is an allergic contact dermatitis or an irritant contact dermatitis. T is condition is seen in patients who play a sport, such as soccer, where a shin guard is commonly used (Fig. 10-23). Some patients have a positive patch test but most do not. T e entity may just represent irritation rom riction and sweating under the shin guard. reatment is aimed at decreasing the in ammation with a topical steroid and protecting the area with a barrier under the shin guard.

SECTION

11 Photodermatoses

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Fig ure 11-1

Fig ure 11-2

Polymorphous light eruption Patients with this condition develop papules, papulovesicles, or erythematous plaques in response to sun exposure. T e lesions erupt a ew hours to several days a er the subject has been exposed to sunlight. Lesions are most o en located on the ace, upper chest, and exposed parts o the extremities. Ocular in ammation and cheilitis may also occur.

Among Native Americans and the indigenous populations o Latin America, polymorphous light eruption tends to be a amilial disease with childhood onset. Figures 11-1 and 11-2 show patients with areas o erythema and edema.

Fig ure 11-3

Fig ure 11-4

Polymorphous light eruption T e “butter y rash” illustrated in Fig. 11-3 gives a sense that polymorphous light eruption can sometimes be di cult to di erentiate rom the cutaneous f ndings in systemic lupus erythematosus. Both immuno uorescence and serology are use ul techniques in di erentiating the two diseases.

Sunscreens, antimalarial medications, topical corticosteroids, and, in older children, psoralens with ultraviolet light (PUVA) are among the treatments used or polymorphous light eruption.

117

Se ction 11 . Photode rm atos e s

Fig ure 11-5

Fig ure 11-6

Photoallergic dermatitis In addition to sunburn and polymorphous light eruption, there are several other abnormal cutaneous reactions in which sunlight is a part o the causative mechanism. In photoallergic dermatitis, sensitization and subsequent clinical reactions develop to a topically applied or internally administered substance that has been activated to allergenicity by sunlight. Sul onamide antibiotics, phenothiazines, and halogenated salicylanilides are among the most common causative agents in photoallergy.

Photoxic dermatitis In phototoxic reactivity, no immunologic mechanism is involved, and the patient reacts as anyone would to a primary irritant. Phototoxic drugs and chemicals include some dyes, coal tar derivatives, and psoralens. Drugs that may cause a phototoxic reaction include the sul onamides, tetracyclines, and thiazides. Pictured in Fig. 11-6 is a teenager who developed erythema on the backs o his hands rom sun exposure while taking doxycycline or his acne.

Fig ure 11-7

Fig ure 11-8

Erythropoietic protoporphyria We have included this metabolic disorder among the photodermatoses because it must always be considered in children who develop a rash a er sun exposure. T is is among the more common porphyrias; onset is usually during early childhood. T e typical presentation o this disease eatures the development o pruritus and burning o the skin in areas o recent sun exposure. T ese sensations may be accompanied by erythema, edema, and vesiculation.

Over time, superf cial scarring and a waxy thickening o the involved areas develop. T ese changes, as seen on the ace, are illustrated in Fig. 11-8. T e bridge o the nose is another common area o involvement. Children with erythropoietic protoporphyria have inherited a def ciency o the enzyme errochelatase, which normally converts protoporphyrin to heme. T e disease can be readily diagnosed by testing or elevated ree erythrocyte protoporphyrin level. T e most serious complication is the development o hepatic involvement. reatment o the skin disease consists o avoidance o the sun and oral therapy with beta-carotene.

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Fig ure 11-9

Fig ure 11-10

NSAID-induced pseudoporphyria Patients being treated with nonsteroidal anti-in ammatory medications (NSAIDs), such as naproxen, oxaprozin, and nabumetone, may experience an eruption characterized by the development o erythema, vesicles, and shallow atrophic scars a er sun exposure.

In many patients, rank vesicles are not appreciated. T e acute eruption may persist or a ew weeks a er the medication has been stopped.

Fig ure 11-11

Fig ure 11-12

Actinic prurigo Actinic prurigo is a chronic skin disorder caused by an abnormal reaction to sunlight. It is most common in indigenous populations in Central and South America. Lesions develop hours or days a er exposure to sunlight, and are pruritic. Many children with actinic prurigo also develop conjunctivitis and swelling and in lammation o the lower lip.

Actinic prurigo cheilitis Cheilitis, particularly o the lower lip, may be the sole mani estation o actinic prurigo. T e swelling may be accompanied by itching, tingling, or pain. reatment o actinic prurigo and o the associated cheilitis is mostly based on various methods o sun protection. opical steroids are also o some benef t and or severe cases, thalidomide is sometimes prescribed.

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Se ction 11 . Photode rm atos e s

Fig ure 11-13

Fig ure 11-14

Hydroa vacciniforme T is is a rare blistering eruption o sun-exposed skin. It most o en occurs during childhood and resolves spontaneously over time. T e itching and burning papules develop within hours a er sun exposure and rapidly progress to vesicles or blisters. T e eyes may also be involved with a mild keratoconjunctivitis.

Some active lesions, and some areas o scarring on the ace are shown in Fig. 11-14. Sun avoidance, the proper applications o high sun protection actor (SPF) sunscreens, and sun protective clothing are strongly advised.

Fig ure 11-15

Fig ure 11-16

Juvenile spring eruption T is term re ers to a disease with its onset in early childhood. Patients develop an intensely pruritic eruption in response to sunlight. T e papules, vesicles, and crusted plaques tend to avor areas o exposed skin but may become generalized. T e disease process is most severe during

the late spring and summer. Illustrated in Figs. 11-15 and 11-16 is the in ammatory pruritic dermatitis on a common location, the pinnae o the ears. reatment or children with this clinical syndrome consists o maximal protection rom sunlight. opical steroids are o some benef t during exacerbations.

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Fig ure 11-17

Fig ure 11-18

Photodermatitis (berloque dermatitis) T is condition is a special kind o photoreaction in which the skin becomes slightly in amed and quickly develops hyperpigmentation. T e cutaneous changes are the result o contact with a photosensitizing chemical, ollowed immediately by sunlight. Figure 11-17 illustrates the orm o this condition that results rom the application o a psoralen-containing per ume, such as oil o bergamot. Note that the hyperpigmentation ollows the exact distribution in which the per ume was applied.

Phytophotodermatitis In addition to per umes, a number o plants, grasses, ruits, and vegetables contain psoralen as a photosensitizer. T e linear lesions pictured Figs. 11-18 and 11-19 resulted rom the dripping o lime juice on to the legs, ollowed by sun exposure.

Fig ure 11-19

Fig ure 11-20

Phytophotodermatitis T e child who helps mother or ather slice limes be ore a trip to the park may develop an identical eruption on the hands. Celery and parsley may present similar problems.

It is important to recognize this entity since some a ected in ants and children have been mistakenly thought to have bruising rom child abuse.

SECTION

12 Papulosquamous Diseases

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Fig ure 12-1

Fig ure 12-2

Seborrheic dermatitis T is term re ers to a scaly, crusting, and erythematous eruption that is most common in in ancy (ages 2-12 weeks), where it tends to avor the scalp, diaper area, and intertriginous olds. Figure 12-1 is an illustration o the process in the scalp where it is o en re erred to as cradle cap.

Figure 12-2 illustrates severe involvement in the eyebrows, a common area o involvement. A subset o in ants with seborrheic dermatitis will go on to develop atopic dermatitis and it sometimes may be di cult to di erentiate between these two conditions.

Fig ure 12-3

Fig ure 12-4

Seborrheic dermatitis Figure 12-3 shows very severe acial and scalp involvement. Some basic principles are that the lesions o seborrheic dermatitis are usually well circumscribed, do not itch, and localize toward the ace, scalp, and intertriginous areas. T e greasy red-orange scaliness o seborrheic dermatitis is somewhat help ul in di erentiating this disorder rom atopic dermatitis.

Figure 12-4 shows a more extensive process that is nearly generalized, dry, and scaly. Seborrheic dermatitis has its onset early in in ancy and usually resolves by 1 year o age; atopic dermatitis tends to be more persistent.

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Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-5

Fig ure 12-6

Seborrheic dermatitis T e cause o this very common condition remains unknown. Although it avors areas with an increased number o sebaceous glands, there is no evidence that seborrheic dermatitis is a disease o sebaceous glands or is related to excessive sebum production.

Some studies have suggested that the lipid composition o sebum in seborrheic dermatitis may be abnormal. Bacteria and yeasts are o en present in areas o involvement, but neither Candida albicans nor Pityrosporum ovale has been shown to be an etiologic agent.

Fig ure 12-7

Fig ure 12-8

Seborrheic dermatitis Seborrheic dermatitis, common during in ancy, is relatively unusual during later childhood. It resur aces as a problem during adolescence and then seems to become progressively more common through adult li e. T e adolescent variant primarily involves the scalp, orehead, tarsal margins o the eyelids (blepharitis), ears, and nasolabial olds. Seborrheic dermatitis is easily controlled but not curable. reatment may consist o the topical application o ketoconazole cream or a mild topical steroid. T e requent use o tar shampoos is particularly help ul in the control o seborrheic dermatitis o the scalp.

During puberty and in adulthood, seborrheic dermatitis occurs not only on the scalp and ace but also on the chest, on the back, and in intertriginous spaces such as the axillae (illustrated in Fig. 12-8), in ramammary areas, groin, and intergluteal olds. Lesions on the chest and back are described as petaloid, that is, at and demarcated like petals; in intertriginous spaces, the appearance can be glistening red.

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Fig ure 12-9

Tinea amiantacea T is term requires etymological explanation. T e tinea does not mean superf cial ungal in ection but rather a condition that resembles a superf cial mycosis. Amiantacea means asbestos-like. T e combination describes a superf cial scaly process that recalls the crumbling ex oliation o asbestos. Such an appearance occurs in the scalp in some cases o seborrheic dermatitis, psoriasis, tinea capitis, and pityriasis sicca (dandru ). T e term is discarded as soon as a better diagnosis is made.

Fig ure 12-10

Fig ure 12-11

Psoriasis More than one-quarter o all individuals with psoriasis develop their disease during childhood or adolescence. T e degree o involvement is extremely variable; some children develop only a ew localized plaques, while others su er rom generalized skin disease and severe arthritis.

Pictured in Figs. 12-10 and 12-11 are the typical lesions o psoriasis; the plaques have a red-to-orange hue, are scaly, and are sharply demarcated rom the surrounding skin.

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Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-12

Fig ure 12-13

Psoriasis T e distinctive character o the scale is appreciated in these f gures. T e scale is usually described as silvery or micaceous (resembling the mineral mica). When the scale is removed, pinpoint areas o bleeding (Auspitz sign) are uncovered.

Each lesion o psoriasis represents an area o rapid epidermal cell turnover. T e thickening o the involved epidermis and the overlying parakeratosis translate into the raised and scaly appearance o the involved skin.

Fig ure 12-14

Fig ure 12-15

Psoriasis T e symmetrical involvement o the knees is a common pattern; elbows and buttocks are other avored locations or plaques like these.

A typical sharply demarcated plaque with micaceous scale on the knee is shown in Fig. 12-14 and on the elbow in Fig.12-15.

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Fig ure 12-16

Fig ure 12-17

Psoriasis In Fig. 12-16 and 12-17, we see plaques o dense adherent scale in the scalp. Circumscribed areas o micaceous scale in the scalp are a common presenting sign o psoriasis. T is disorder can usually be di erentiated rom tinea capitis by the character o the scale, the sharp demarcation, and, usually, the absence o hair loss.

Lesions in the scalp tend to cause pruritus. When treating the scalp, it is important to decrease the scale so that the topical medications become more e ective. Preparations containing salicylic acid, a keratolytic, are especially use ul or the treatment o scalp psoriasis.

Fig ure 12-18

Fig ure 12-19

Psoriasis In Fig. 12-18, we see typical involvement o the glabrous skin o the neck, with extension into the scalp. T is is a common location, and, in some cases, psoriasis may develop over a persistent nevus simplex (stork bite) in that area.

Unilateral, or, more commonly, symmetrical involvement o the skin around the eyes may occur in psoriasis. T e involvement o the medial aspect, as shown in Fig. 12-19, is particularly common.

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Fig ure 12-20

Fig ure 12-21

Psoriasis Figures 12-20 to 12-23 show the erythema, scaling, and thickening o portions o the palms and soles that are very common in both children and adults with psoriasis. T erapy o psoriasis is based on the skill ul use, either alone or in combination, o a number o therapeutic agents.

T e most common topical treatments include topical steroids, tars, keratolytics, ultraviolet light, and topical calcipotriol and tazarotene in older patients. Children with simple plaque psoriasis can sometimes be managed with short-contact anthralin preparations.

Fig ure 12-22

Fig ure 12-23

Psoriasis When topical steroids are used, it is important to employ the least potent preparation that is e ective and to avoid the use o uorinated steroids on the ace and in intertriginous areas. Care ul exposure to sunlight during the summer months and artif cial ultraviolet light at other times is enormously benef cial in selected patients with extensive involvement.

T ickening and f ssuring o the palms or soles can become extremely pain ul. Patients with severe involvement like this, and those with severe generalized involvement may sometimes require systemic therapy. reatments include methotrexate, acitretin, and biologic agents.

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Fig ure 12-24

Fig ure 12-25

Psoriasis In Fig. 12-24, the lesions consist o numerous papules, each covered with the typical silvery scales o psoriasis. T is orm o the condition, termed guttate psoriasis, is more common in childhood and may have an explosive onset. T ere is o en a history o an antecedent upper-respiratory in ection, and streptococcal disease is o particular importance in triggering this eruption. T e use o oral antibiotics that are e ective against Streptococcus sometimes hastens the resolution o guttate psoriasis. T e use o mild topical corticosteroids is o benef t.

Rarely, patients develop psoriasis in a linear distribution. In some cases, this is a simple result o Koebnerization, the development o lesions in areas that are being scratched or traumatized in some other way. In other cases, the development o linear psoriasis ollows the lines o Blaschko, and may indicate a somatic mutation in the skin.

Fig ure 12-26

Fig ure 12-27

Psoriasis Pictured here are two representations o psoriasis in the diaper area. In Fig.12-26, the entire diaper area is involved with a sharply demarcated erythematous scaling eruption. Figure 12-27 shows the sharply demarcated eruption with minimal involvement o the scrotum but involvement o the prepuce o the penis. When onset o the disease occurs during in ancy, this is a very common area o involvement.

It is postulated that the repeated irritation in this area constitutes a type o Koebner phenomenon. Scales are less in evidence in Fig. 12-26 because o the maceration that is inevitable in this location. Note the sharp demarcation o the lesions. reatment o psoriasis in the diaper area can be di cult. Low-potency topical corticosteroids should be used judiciously with the use o barrier creams.

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Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-28

Fig ure 12-29

Pustular psoriasis Figures 12-28 and 12-29 illustrate pustular psoriasis. Both are examples o the disease in relatively mild orm, but even in these the suppurative quality o the lesions can be appreciated. Severe pustular psoriasis, also known as the von Zumbusch form, is a rare and potentially li e-threatening disease.

Pustular psoriasis may be triggered by physical or emotional stress, a number o medications, or the abrupt discontinuation o steroid therapy, and must be di erentiated rom other disorders, including pustular drug eruptions.

Fig ure 12-30

Fig ure 12-31

Pustular psoriasis Patients with this orm o disease can develop shaking chills, ever, and leukocytosis. Numerous superf cial pustules develop on psoriatic plaques and on uninvolved skin. Over a brie period o time, the pustules enlarge and become con uent; lakes o pus orm. T e process may eventuate in an ex oliative erythroderma. Hospitalization and care ul supportive therapy are important aspects o treatment.

Pustules with identical appearance may be seen in a def ciency o interleukin 1 receptor antagonist (DIRA), and this disorder may also cause lytic lesions in the bones. reatment or this disorder is anakinra, a recombinant orm o the human interleukin-1 receptor antagonist.

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Fig ure 12-32

Fig ure 12-33

Pityriasis rubra pilaris T is is a chronic and o en severe cutaneous disorder that may sometimes begin during childhood. Depending on the stage and location o the disease process, the appearance varies. T e most unique distinguishing mani estation o this disease is the red-orange peri ollicular keratotic papules that are usually located on the dorsal sur aces o the f ngers and hands. T e “nutmeg grater” appearance in these areas is pathognomonic o pityriasis rubra pilaris.

Figures 12-32 and 12-33 illustrate the disease process as it appears on the trunk. Follicular localization may be apparent early in the condition, but later, orange-red plaques with scaling are more usual. T e condition may become generalized, but usually some islands o normal-appearing skin remain interspersed between involved areas.

Fig ure 12-34

Fig ure 12-35

Pityriasis rubra pilaris Figure 12-34 and 12-35 show both discrete ollicular papules and larger con uent, psoriasi orm plaques on the leg.

T e circumscribed collection o scaly papules, which become con uent, is characteristic, but may, at times, be di cult to di erentiate rom psoriasis.

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Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-36

Pityriasis rubra pilaris Erythema and scaling o the ace and scalp may represent the initial presentation o pityriasis rubra pilaris. Pityriasis rubra pilaris in children has been divided into subtypes based on natural history and clinical appearance. T e classic juvenile orm a ects children in the f rst 2 years o li e and o en tends to become generalized and severe. T e circumscribed juvenile type presents with patches o ollicular papules and displays a lesser tendency toward progression to generalized disease. Finally, the atypical juvenile orm is the rarest, has the poorest prognosis, and tends to be amilial.

Fig ure 12-37

Figure 12-37 illustrates the highly characteristic appearance o the condition on the palm, knee, and sole. T ere is a di use hyperkeratosis that is symmetrical and covers the entire palmar sur ace. Dystrophy o both f ngernails and toenails may be prominent. Palmoplantar involvement in pityriasis rubra pilaris is very common and can be disabling.

Fig ure 12-38

Fig ure 12-39

Pityriasis rosea T is benign and sel -limited eruption occurs most o en in spring and autumn. Most patients are adolescents and young adults, but the disorder is not unusual in children and may even occur during in ancy. In its classic orm, pityriasis rosea ollows a specif c and predictable clinical course. T e f rst solitary lesion is a circle or oval o erythema and scaling. As it develops to its ull size o up to 2 to 3 cm, this so-called herald patch may easily be mistaken or a lesion o tinea corporis. T e chest and upper thigh are common locations or the herald patch but any area may be involved.

A typical herald patch is shown in Fig. 12-38 and the lower edge o Fig. 12-39. Within a period o 5 to 15 days, additional lesions begin to develop. Patients develop numerous round-to-oval pink-orange macules and papules that are 3 to 10 mm in diameter. Each has a trailing edge o f ne scaling, the characteristic “collarette.” Larger, round-to-oval patches may also be present. T e lesions o pityriasis rosea tend to cluster on the trunk and proximal extremities and o en are most numerous in the axillae. T e generalized process is illustrated in Fig. 12-39. T e duration o the total process is 6 to 9 weeks.

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Fig ure 12-40

Fig ure 12-41

Pityriasis rosea Figures 12-40 and 12-41 illustrate well-developed lesions o pityriasis rosea in the midcourse o the condition. One can appreciate the parallel array o the individual macules. On the back and chest, this tendency o the lesions to ollow skin lines (the so-called Christmas tree distribution) is usually most obvious.

A number o atypical orms o pityriasis may occur and these variations in both morphology and distribution seem to be more common during childhood. In particular, papules, pustules, and even vesicles may occur and their presence may suggest a number o other cutaneous disorders. However, a care ul search will usually lead to one or several o the typical papulosquamous lesions. In addition, lesions may extend to involve the ace and there may be relative sparing o the trunk. In some cases, the process is conf ned to intertriginous areas, as seen in Fig. 12-41.

Fig ure 12-42

Fig ure 12-43

Pityriasis lichenoides chronica Pityriasis lichenoides chronica is a chronic disorder that consists o superf cial lesions that evolve rom papules into oval pink-brown papulosquamous lesions. T ere may be an adherent scale overlying the individual lesions, and there is sometimes mild pruritus. Involvement tends to avor the trunk and proximal extremities, although it may become more widespread.

Many cases o pityriasis lichenoides chronica are initially diagnosed as pityriasis rosea. However, the persistent course o this disease, with remissions and exacerbations, eventually distinguishes it. Pictured in Fig. 12-43 is a child with both crusted papules and hypopigmented macules. Many children with pityriasis lichenoides chronica develop areas o hypopigmentation, and these may arise de novo on normal skin.

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Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-44

Fig ure 12-45

Pityriasis lichenoides chronica T e widespread areas o hypopigmentation in this disorder may be persistent and disf guring, involving also the ace, as shown in (Fig. 12-45). In most cases, treatment with topical agents is o little value, and the most improvement is seen with narrow band ultraviolet B-light therapy. I this is unavailable, sun exposure may be help ul.

In our opinion, the relationship between this orm o hypopigmentation and hypopigmented mycosis ungoides remains unclear. We do believe that children with this clinical phenotype usually show a good response to light treatment, and overall have an excellent prognosis.

Fig ure 12-46

Fig ure 12-47

Pityriasis lichenoides et varioliformis acuta (PLEVA, MuchaHabermann disease) T is is a troubling papulosquamous disorder o acute onset that occurs in both children and young adults. Involvement tends to avor the anterior trunk and proximal extremities. Facial and palm and sole involvement is relatively rare, and pruritus is usually absent. Involvement o the axillae, as shown here, is common.

T e individual lesions go through a distinctive process o evolution over a period o weeks. Lesions tend to occur in crops. Many patients are mistakenly labeled as having chickenpox; however, the typical dew drop on a rose petal lesion is not seen in PLEVA and the course o the disease is entirely di erent.

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Fig ure 12-48

Fig ure 12-49

Pityriasis lichenoides et varioliformis acuta (PLEVA, MuchaHabermann disease) Each lesion begins as an erythematous papule. T e lesion enlarges to become a brownish 2 to 3 mm oval papule and then can develop a central area o hemorrhagic or necrotic crust. Lesions o this type are depicted in Figs. 12-48 and 12-49.

T e condition generally has no systemic mani estations and tends to resolve gradually over a period o 4 to 6 months. However, some childhood cases last considerably longer and scarring and pigmentary changes requently occur.

Fig ure 12-50

Fig ure 12-51

Pityriasis lichenoides et varioloformis acuta (PLEVA, MuchaHaberman disease) Pictured in Fig. 12-50 is a child with numerous crusted papules on the abdomen. T ere is some evidence that resolution o this process can be hastened by a course o oral erythromycin or tetracycline (over age 9). T e mechanism o action o the drug in this situation is not known.

Figure 12-51 illustrates a severe case o PLEVA. Rarely, patients may develop a variant that is characterized by ulceronecrotic lesions, ever, and systemic symptoms.

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Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-52

Fig ure 12-53

Lichen nitidus T is is an unusual and distinctive dermatosis that has its peak incidence during childhood. T e individual papules, as pictured in Fig. 12-52, are smaller than 1 mm in diameter. T ey are at and shiny, with a round or polygonal shape.

T e lesions usually cluster, and an occasional linear grouping suggest that they occur in areas o trauma (the Koebner phenomenon) (Fig. 12-53). Lichen nitidus is occasionally seen in association with lichen planus. However, the marked di erences in both histopathology and natural history indicate that they are di erent diseases.

Fig ure 12-54

Fig ure 12-55

Lichen nitidus In Fig. 12-54, the shiny character o the lesions o lichen nitidus is more appreciable. T e lesions o lichen nitidus are usually asymptomatic but pruritus may occur.

T e penis is a site o predilection or this condition, as are the abdomen and upper extremities. T ey may clear spontaneously in a short period o time or they may last or months or years. T ere is no known e ective treatment or the condition itsel ; itching can be treated symptomatically with antihistamines and mild topical steroids.

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Fig ure 12-56

Fig ure 12-57

Lichen striatus T is is a common and benign sel -limited childhood dermatosis that is easily diagnosed rom its classic appearance. Onset is usually between the ages o 3 and 10 years.

Lichen striatus is rare in young in ants, adolescents, and adults. T e lesions consist o pink, esh-colored, or slightly hypopigmented at-topped papules that evolve in a linear array ollowing lines o Blaschko.

Fig ure 12-58

Fig ure 12-59

Lichen striatus Figure 12-58 illustrates a typical lesion o lichen striatus occurring in a di erent acial location. T e area o involvement is o en noted to become wider as it advances.

Figure 12-59 demonstrates lichen striatus ollowing Blaschko lines on the trunk. T e lesions o lichen striatus are usually asymptomatic but may last rom months to years.

137

Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-60

Fig ure 12-61

Lichen striatus T e linear array o papules in this patient involves the entire arm. Because o the distribution along Blaschko lines, it is believed that lichen striatus represents in ammation o a group o cells which di erentiated due to a somatic mutation during embryogenesis.

Postin ammatory hypopigmentation occurs ollowing the initial in ammatory process. T e etiology o the condition is unknown. reatment is not strictly necessary, but mild topical steroids tend to speed the process o resolution.

Fig ure 12-62

Fig ure 12-63

Lichen striatus Figure 12-62 illustrates another linear lesion o lichen striatus, in a typical lower extremity distribution.

Lichen striatus may extend along the f nger and then cause dystrophy o the associated f ngernail or toenail. In some cases, distortion or destruction o the nail may be permanent.

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Fig ure 12-64

Fig ure 12-65

Follicular mucinosis T is is an uncommon disorder o unknown etiology that a ects both children and adults. T e lesions, which are usually located on the head and neck, vary in clinical appearance. Most o en, as illustrated in this f gure, there are grouped esh-colored papules.

T is f gure shows a patient with an erythematous scaly plaque and loss o eyebrow hair. When the disorder a ects the scalp, it causes areas o permanent alopecia. In children, this disease tends to resolve spontaneously. Rarely, however, an association with lymphoma may occur. T is can usually be diagnosed by care ul examination o a skin biopsy at the time o disease onset.

Fig ure 12-66

Fig ure 12-67

Follicular mucinosis In some cases, as illustrated in Fig. 12-66, there may also be erythematous plaques with ollicular accentuation and scale. In this patient, the areas o involvement resolved with ollicular atrophy, as illustrated in the second igure (Fig. 12-67).

In some children, only a ew lesions develop and the condition resolves spontaneously over time. However, in others, the disorder is persistent and new lesions continue to develop.

139

Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-68

Fig ure 12-69

Porokeratosis T is is a rare autosomal dominant dermatosis with unique clinical and histologic f ndings. Lesions usually develop during childhood and avor the hands, orearms, and ace. Each lesion begins as a papule and evolves into an irregular atrophic plaque. A well-developed plaque is pictured in Fig. 12-68.

In Fig. 12-69 one can begin to appreciate the raised hyperkeratotic border that surrounds each circinate plaque. T is border, which corresponds to the histologic f nding cornoid lamella, is the most diagnostic clinical eature.

Fig ure 12-70

Fig ure 12-71

Porokeratosis (porokeratosis of Mibelli) Pictured in these f gures are two variants o porokeratosis. T e f rst is an isolated lesion, sometimes re erred to as porokeratosis of Mibelli. Note the raised hyperkeratotic border and circinate plaque.

Linear porokeratosis T e second case shows a linear array o lesions on the thigh. Linear porokeratosis, which ollows the lines o Blaschko, is probably due to somatic mutation and resultant chromosomal mosaicism.

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Fig ure 12-72

Fig ure 12-73

Elastosis perforans serpiginosa T is rare disorder is characterized by small, cone-shaped, hyperkeratotic papules that are arranged in annular or circinate patterns. he lesions are usually localized but the process may also be disseminated. T e underlying histologic process is the transepidermal elimination o elastic tissue.

An association with heritable disorders such as Ehlers-Danlos syndrome, Mar an syndrome, pseudoxanthoma elasticum, osteogenesis imper ecta, Rothmund-T omson syndrome, and Down syndrome has been reported. In most patients, there is no e ective therapy. Attempts at surgical removal are complicated by the high incidence o recurrence and scar ormation.

Fig ure 12-74

Fig ure 12-75

Perforating folliculitis T is eruption consists o numerous small erythematous ollicular papules with central keratotic plugging. T e lesions, which vary in size rom 2 to 8 mm, are usually located on the buttocks and thighs. T e cause o this eruption is unknown; irritation o the hair ollicle is probably the primary process. At various times, per orating olliculitis has been ascribed to the wearing o tight garments or to some chemical in clothing.

Reactive perforating collagenosis T is rare disease usually has its onset during in ancy and early childhood. Autosomal recessive, dominant, sporadic, and acquired orms have been reported. T e lesions, as shown in Fig. 12-75, consist o small erythematous papules that gradually increase in size and develop a central hyperkeratotic plug. In some cases, the linear array o lesions suggests that they are induced by trauma—the so-called Koebner phenomenon. Pruritus is an occasional eature. Lesions usually last 6 to 8 weeks and then resolve, only to be ollowed by the eruption o resh papules.

141

Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-76

Fig ure 12-77

Lichen planus T is condition is a pruritic eruption o unknown etiology. It is not uncommon in childhood. T ese illustrations are o the most representative lesions o lichen planus on a most common site, the wrists. T e primary lesion consists o a attopped, polygonal, violaceous papule 2 to 6 mm in diameter. T e characteristic shiny appearance o the individual papules is seen in these f gures.

Figure 12-77 illustrates the tendency or the solitary lesions to orm con uent plaques. Exaggerated sur ace markings in the overlying skin (Wickham striae) may also be evident but are di f cult to appreciate rom these f gures. T e orearms, the middle o the back, and the anterior sur aces o the lower extremities are other common locations.

Fig ure 12-78

Fig ure 12-79

Lichen planus T e clinical severity o lichen planus varies rom a ew mildly pruritic lesions in some cases to extensive and severe involvement o the skin and mucous membranes with intractable itching in others. Children with limited disease o en respond well to the local application o topical corticosteroids. However, when the process is generalized, a short course o systemic steroids may be necessary and will sometimes yield a dramatic improvement.

On the legs, lesions may become markedly hypertrophic and plaque-like. Lichen planus tends to be a problem o long duration, with periods o remission and exacerbation.

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Fig ure 12-80

Fig ure 12-81

Lichen planus Figure 12-80 illustrates the tendency or new lesions to orm in a scratch or abrasion. T e so-called Koebner phenomenon is highly characteristic o lichen planus, and some evidence o “Koebnerization” is seen in almost every patient with this disorder.

Most o en, the lesions o lichen planus are small purplish papules, solitary or con uent, with exaggerated sur ace markings. T ere are, however, several variants. Some lesions develop adherent scales, sometimes vesiculation occurs, and rarely, necrosis and scarring may occur upon resolution. T e lesions in Fig. 12-81 are larger and more in ammatory than usual; there is a suggestion o vesiculation and necrosis. T e vesicular and bullous orms o lichen planus must sometimes be di erentiated rom other bullous disorders.

Fig ure 12-82

Fig ure 12-83

Lichen planus Although the etiology o lichen planus remains a mystery, the clinician must bear in mind that certain lichenoid drug eruptions may be clinically indistinguishable rom true lichen planus. T e most common agents are gold salts and antimalarial agents. opical exposure to paraphenylenediamine may have the same result.

More than one-hal o patients with cutaneous lesions will have oral mucosal involvement, and mucosal involvement may also occur without any skin lesions. T e oral lesions are most commonly ound on the buccal mucosa and the lips. T e lesions are characteristically white reticulated patches, although bullae, erosions, and ulcers may be seen. Erosive lesions may be quite pain ul.

143

Se ction 1 2 . Papulos quam ous Dis e as e s

Fig ure 12-84

Fig ure 12-85

Lichen planus A small percentage o patients with lichen planus develop nail involvement. T e severity varies. Some children develop only mild thinning or ridging o the nail plate. Others have a severe nail dystrophy, with pterygium ormation and complete and permanent nail loss.

A case where the nails are destroyed is pictured in Fig. 12-85. Rarely, there may even be severe lichen planus o the f ngernails and toenails without skin involvement. In any case, attempts to treat the severe orms o nail disease in lichen planus are rarely success ul.

Fig ure 12-86

Fig ure 12-87

Actinic lichen planus T is rare orm o lichen planus occurs on sun-exposed areas and consists o either annular lesions, or, as pictured in Fig. 12-86, violaceous papules which coalesce to orm plaques. opical corticosteroids and antimalarials are among the e ective treatments. Patients must also use topical sunscreens.

Annular lichen planus Figure 12-87 shows a patient with annular lesions on the ace. T is presentation is rare and overlaps with actinic lichen planus, in which the disease occurs on sunexposed areas, most o en the ace. T e lesions are usually round and well-def ned. A central area o hyperpigmentation is surrounded by either erythema or hypopigmentation. T e majority o cases seem to occur in India, A rica, and the Middle East.

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Fig ure 12-88

Fig ure 12-89

Lichen planopilaris (follicular lichen planus) T is is a variant o lichen planus in which the primary involvement occurs around hair ollicles. It is more common in emales. Patients with this orm o disease may have the more typical at-topped papules and mucosal lesions as well. Pictured in Fig. 12-88 are numerous rough ollicular papules on an extremity.

Lichen planopilaris can be a severe and disf guring disorder when it involves the scalp. In such cases, either a temporary or permanent scarring alopecia may develop. Figure 12-89 shows a scalp with a scarring alopecia, ollicular spines, and erythema. T is condition is progressive and may be very di cult to treat, although the use o potent topical corticosteroids may be use ul.

SECTION

13 Nutritional, Metabolic, and Endocrine Diseases

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Fig ure 13-1

Fig ure 13-2

Acrodermatitis enteropathica T is syndrome results rom inadequate absorption or dietary intake o zinc. Figure 13-1 shows erythema, crusting, and ssuring o the perioral skin and cheeks. T e eruption that is pictured here may be preceded by blisters. Other eatures o acrodermatitis enteropathica include stomatitis, paronychia, and alopecia.

T e diaper area lesion that is seen in Fig. 13-2 is di usely erythematous and has a sharply marginated border on the abdomen. Acrodermatitis enteropathica may be inherited in an autosomal recessive ashion. T is orm o the disease seems to be related to an inability to absorb zinc.

Fig ure 13-3

Fig ure 13-4

Acrodermatitis enteropathica Figure 13-3 shows a highly characteristic picture o the cutaneous changes o acrodermatitis enteropathica around the anus, the buttocks, and on the perineum. Note the psoriasi orm appearance o this lesion and o those on the eet in Fig. 13-4. T e ull-blown picture o acrodermatitis enteropathica goes ar beyond the typical changes o skin and hair. A ected children have severe diarrhea, growth

retardation, and irritability. Without treatment, the disease ollows a progressive course and may even be atal. T e child with suspected acrodermatitis enteropathica should be evaluated or a low zinc level or a low alkaline phosphatase level when zinc levels are normal or below normal. reatment with dietary zinc supplementation leads to a dramatic resolution o all symptoms and, in some cases, must be maintained inde nitely.

Se ction 1 3 . Nutritional, Me tabolic, and Endocrine Dis e as e s

147

Fig ure 13-5

Fig ure 13-6

Acrodermatitis enteropathica Acquired acrodermatitis enteropathica is seen in in ants who have received parenteral alimentation lacking su cient zinc and, rarely, in breast- ed premature in ants who have larger zinc requirements. Occasionally, acrodermatitis enteropathica in a ull-term breast- ed in ant may be the result o low levels o zinc in the breast milk.

T e patient with acquired acrodermatitis enteropathica requires temporary zinc replacement. T e di erential diagnosis o this eruption includes psoriasis, biotin and multiple carboxylase de ciencies, essential atty acid de ciencies, and cystic brosis.

Fig ure 13-7

Fig ure 13-8

Kwashiorkor Kwashiorkor is a type o protein energy malnutrition. It is seen most commonly in developing countries, and onset tends to occur a er weaning. At that time, the balance o protein and carbohydrate in breast milk is replaced by a diet that contains almost exclusively carbohydrates.

T e initial signs are diarrhea, irritability, and edema o the hands and eet. Small dark patches appear at pressure points o the elbows, ankles, wrists, and knees, and then spread. T e patches have a sharp border and tend to peel; the super cial desquamation in these areas is o en likened to the appearance o f aking paint or enamel.

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Fig ure 13-9

Fig ure 13-10

Kwashiorkor As the condition progresses, there develops a generalized red-brown discoloration. Other ndings include ssuring at the edges o the mouth (Fig. 13-9) and the development o coarse, hypopigmented hair. Photosensitivity and easy bruising may also be present.

Marasmus Figure 13-10 illustrates the classic “baggy pants” appearance in protein-calorie malnutrition, also known as marasmus. Due to prolonged starvation, the child appears very thin, and has little subcutaneous at or muscle mass. T e child may also have a thin “old man” ace. T ere is no associated edema o the lower extremities.

Fig ure 13-11

Fig ure 13-12

Pellagra Pellagra is a disease caused by inadequate dietary intake o niacin. It is now seen in parts o the world where dietary intake o tryptophan, an amino acid precursor o niacin, is inadequate. In particular, diets that consist largely or exclusively o maize or millet predispose to this disease. T e signs and symptoms o pellagra are o en remembered by the mnemonic our Ds: dermatitis, diarrhea, dementia, and death. T e classic cutaneous changes are inf ammation, hyperpigmentation, and

scaling in symmetric distribution and in areas exposed to heat or sunlight. ypical areas o involvement are the hands and orearms, legs and eet, and ace and neck. Figure 13-11 shows moderate changes on the eet and legs. Figure 13-12 shows the scaling dermatitis on the ace and an angular cheilitis. Edema and inf ammation o the tongue are also common eatures o pellagra. T e addition o supplemental niacin to the diet brings a quick resolution to the disease.

Se ction 1 3 . Nutritional, Me tabolic, and Endocrine Dis e as e s

149

Fig ure 13-13

Fig ure 13-14

Lipoid proteinosis T is is a rare autosomal recessive disease that is caused by the deposition o hyaline material in the skin and mucous membranes. Laryngeal involvement may be present rom birth and eventually produces a characteristic hoarseness in every a ected individual. Cutaneous disease begins during the rst 2 years o li e and consists o papules, nodules, and areas o thickening and hyperkeratosis.

T e cutaneous lesions on the alae nasi and in the choanae are shown in Fig. 13-13. In Fig. 13-14, the mucosal sur ace o the lower lip is extensively involved with the characteristic papules. Not shown here are the numerous small papules that dot the ree margins o the eyelids. T e disorder is due to a de ect in the extracellular matrix protein 1 gene mapped to chromosome 1q21.

Fig ure 13-15

Fig ure 13-16

Lipoid proteinosis Pictured in Fig. 13-15 are a plaque and lesions o smaller sizes on and around the elbow. Most children with lipoid proteinosis continue to develop additional lesions during adult li e. Lesions in the mouth and oropharynx cause the most serious sequelae. T e tongue may become thick and bound down, and dysphagia and respiratory obstruction may result rom lesions in the pharynx and larynx. Finally, intracranial calci cations are a common eature o lipoid proteinosis. For most patients, these are asymptomatic but seizure disorders may occur.

Hurler syndrome T is inherited condition results in the accumulation o chondroitin sul ate B in the skin and other organ systems. Dwar sm and an unusual acial appearance are also aspects o the disease. Figure 13-16 is an excellent representation o shagreen skin, an appearance that is also seen in tuberous sclerosis. Shagreen re ers to a type o leather that is embossed with knobs by processing and then variably stained.

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Fig ure 13-17

Fig ure 13-18

Xanthomatosis Xanthomas are papules or nodules o the skin or mucous membranes that contain lipids. T e appearance o xanthomas during childhood should prompt a thorough search or underlying systemic disease. T e yellowish papules seen in Fig. 13-17 are a orm o planar xanthoma. T ese may occur on any part o the body and may be an indicator o a hereditary lipoproteinemia, diabetes mellitus, or liver disease. Multiple myeloma and Langerhans cell histiocytosis are less common etiologies. T e patient pictured here has biliary cirrhosis.

T e lesions illustrated in Fig. 13-18 are typical o xanthoma striatum palmare, a orm o planar xanthoma. T is patient also has biliary cirrhosis. Note how the yellowish papules and plaques ollow the creases o the ngers and the palmar olds. T e amilial hyperlipidemias, particularly types II, III, and V, may present with an identical clinical picture. Patients with these disorders are at high risk or ischemic heart disease.

Fig ure 13-19

Fig ure 13-20

Xanthomatosis T ese small papules on the dorsa o the eet are also xanthomas. T e patient is a 4-year-old child with type II hyperlipoproteinemia. T is is an autosomal dominant condition in which there may be massive elevations in serum cholesterol. Individuals with this disease o en develop ischemic heart disease during young adulthood. T e recognition o the cutaneous lesions is important in identi ying children who may require dietary or medical management o their hypercholesterolemia.

T ese yellow-red nodules shown in Fig. 13-20, which o en occur on the elbows and knees, are termed tuberous xanthomas. T ey may also be ound in other areas where ordinary trauma is common, or example, buttocks, knuckles, and heels. Similar lesions that overlie extensor tendons are sometimes called tendon xanthomas. Lesions o this sort are almost always caused by a hyperlipoproteinemia and should prompt investigation o serum cholesterol and triglycerides. ypes II, IV, and V are those most commonly associated with tuberous and tendon xanthomas.

Se ction 1 3 . Nutritional, Me tabolic, and Endocrine Dis e as e s

151

Fig ure 13-21

Fig ure 13-22

Xanthomatosis Not all xanthomatoses are rooted in abnormalities o cholesterol metabolism or other systemic disease. In Fig. 13-21, xanthomatous papules that developed in a lymphedematous leg are shown. Since serum lipids are normal, local causes stemming rom the lymphatic obstruction must account or the lesions. T e condition does not have the serious import o those xanthomatoses associated with abnormalities o serum lipids.

Calcinosis cutis When calci cation occurs in the skin, it may represent an isolated local event, or it may be a sign o an underlying systemic disease. T e lesion pictured in Fig. 13-22 is a solitary nodular calci cation. T ese sometimes result rom local trauma, such as an insect bite, or rom the rupture o an epidermal cyst (dystrophic calcinosis cutis). When such nodules occur on the ace o an in ant, they are usually idiopathic and o no medical signi cance.

Fig ure 13-23

Fig ure 13-24

Calcinosis cutis T e nodular calci cations pictured in Fig. 13-23 also turned out to be idiopathic. However, grouped calci cations such as these may also be seen in children with CRS syndrome (calcinosis cutis, Raynaud phenomenon, sclerodactyly, telangiectasia) or dermatomyositis. Pseudoxanthoma elasticum and Ehlers-Danlos syndrome are other causes. T e so-called metastatic calcinosis cutis, with widespread precipitation o calcium salts, is a sign o abnormal calcium metabolism and may result rom parathyroid tumors, chronic renal ailure, or vitamin D intoxication.

Progressive osseous heteroplasia In this rare disorder, bone orms within skin and muscle tissue. Ectopic bone ormation starts in the dermis and subcutaneous at and gradually extends into other tissues such as tendons and skeletal muscle. Progressive osseous heteroplasia is caused by a mutation in the GNAS gene.

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Fig ure 13-25

Fig ure 13-26

Acanthosis nigricans T is common dermatosis is characterized by velvety, brownish-black plaques. T e most common area o involvement is the neck, as pictured in Figs. 13-25 and 13-26, and the second most common is the axilla. Lesions also occur on f exural areas o the elbows, knees, and groin, and on the dorsal hands, especially over the ngers.

In children and adolescents, acanthosis nigricans is usually associated with obesity and insulin resistance. It o ers a clue to either the diagnosis o type 2 diabetes or or the need or diet and exercise in order to prevent the development o this disease.

Fig ure 13-27

Fig ure 13-28

Acanthosis nigricans In some patients, the color and texture o the lesions will improve with the application o topical retinoids. Importantly, the diagnosis o acanthosis nigricans can be seen as an opportunity to counsel overweight and obese patients about diet and physical activity. Patients with hypertension, hypercholesterolemia, hypertriglyceridemia, or elevated asting glucose may require medical therapy.

Very rarely, acanthosis nigricans develops as a sign o malignancy. It may also occur as a side e ect o medications, including niacin, oral contraceptives, and protease inhibitors. Acanthosis nigricans may also be a mani estation o a variety o syndromes, including Costello syndrome and MORFAN syndrome.

SECTION

14 Genodermatoses

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Fig ure 14-1

Fig ure 14-2

Pseudoxanthoma elasticum T is is a generalized condition in which elastic bers are degenerative. Clinical signs o the phenomenon can be recognized in the skin and eyes. In the skin, patches o yellowish discoloration and general laxness or redundancy develop on the neck (“chicken skin”), in the axillae, and in other places, such as the ossae o limbs and the inguinal olds,

where considerable movement o skin is normal. In the eye, angioid streaks can be seen. T ey represent the result o aulty elastic bers in Bruch membrane and generally precede the cutaneous changes. T ese eye changes requently result in the loss o central vision and sometimes result in almost complete blindness. Peripheral vision is maintained.

Fig ure 14-3

Fig ure 14-4

Pseudoxanthoma elasticum Gastrointestinal hemorrhage is the most serious acute consequence, but slower structural damage in various organs may result in hypertension, coronary artery occlusion, diabetes mellitus, thyroid dyscrasia, or ectopic calcinosis. T e disease may be inherited in autosomal recessive or autosomal dominant ashion. T is entity is caused by mutations in the ABCC6 gene mapped to chromosome 16p13.1.

Cutis laxa T is disorder may be caused by mutations in genes that are responsible or the ormation, assembly, or unction o elastic bers, or may be later in li e and related to the destruction o elastic bers. As seen in this patient, the skin hangs in olds and produces an appearance o premature aging. Because elastic bers are a ected in all organ systems, intestinal and urinary bladder diverticula, rectal prolapse, inguinal hernias, and pulmonary emphysema occur requently. T e last o these is associated with signi cant mortality.

155

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-5

Fig ure 14-6

Ehlers-Danlos syndrome T is syndrome is actually a collection o six major genetic types with the common eatures o hyperextensible skin and joints, easy bruising, de ective wound healing, and blood vessel ragility. Distinct abnormalities in collagen synthesis have been identi ed in some o the varieties o Ehlers-Danlos syndrome. T e result o the anomaly is extreme

stretchability but unimpaired elasticity (ie, the ability to return to normal a er stretching). T e gures illustrate the phenomenon; Fig. 14-5 shows skin o the neck and Fig. 14-6 shows skin o the elbow extended several times more than normal skin can be pulled out.

Fig ure 14-7

Fig ure 14-8

Ehlers-Danlos syndrome In these illustrations, more o the hyperextensible phenomena and the consequences o unctional abnormality o elastic bers and collagen are shown. Figure 14-7 shows hyperextensibility o joints, rom which it may be in erred that skin, ligament, tendon, and to some extent bone are also abnormally stretchable. Another way in which so tness o muscle and related structures can be appreciated is in the eel

o a handshake with a patient who has Ehlers-Danlos syndrome. No matter how hard one presses, there is a eeling that one is not quite through with the handshake. Figures 14-8 illustrates the result o incisions and shearing trauma in the skin o a patient with Ehlers-Danlos syndrome. T e result is hemorrhage, ailure o healing by primary intention, and nally broad, riable scars.

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Fig ure 14-9

Fig ure 14-10

Ehlers-Danlos syndrome Small “pseudotumors” on easily traumatized areas such as the elbows and knees, illustrated in Figs. 14-9 and 14-10, are actually subcutaneous lesions that develop rom brosis or calci cation o hematomas.

Fat-containing cysts that become calci ed, known as spheorids, are usually ound on the orearms and shins. Depending on the type o Ehlers-Danlos, inheritance may be autosomal dominant, autosomal recessive, or X-linked recessive.

Fig ure 14-11

Ehlers-Danlos syndrome Gastrointestinal per oration and rupture o a large artery are the most severe complications o some orms o this syndrome. Premature birth (probably due to ragility o the etal membranes) is a common event in patients with Ehlers-Danlos syndrome. Figure 14-11 shows another example o the dis guring scars that are seen in this condition.

157

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-12

Fig ure 14-13

Focal dermal hypoplasia (Goltz syndrome) T is syndrome is caused by heterozygous mutation in the PORCN gene on chromosome Xp11.23. T e syndrome is transmitted in an X-linked dominant ashion. T e most common cutaneous lesions are linear or reticulate areas o hypoplasia with telangiectasia, atrophy, and abnormal pigmentation. Figure 14-12 shows some lesions o this type, as well as the nodular at tumors that are typically present.

Figure 14-13 shows the whorled nature o the lesions on the trunk ollowing the lines o Blaschko. Ulcers may be present initially in the areas o congenital absence o skin and heal with atrophy. T e range o clinical presentation varies rom minor involvement on the limbs to extensive distortion o the skin and bony skeleton.

Fig ure 14-14

Fig ure 14-15

Focal dermal hypoplasia (Goltz syndrome) When bone is involved, syndactyly, polydactyly, oligodactyly with lobster claw de ormity (as seen in Fig. 14-14), skeletal asymmetry, and scoliosis may occur. Ocular abnormalities include colobomas, microphthalmia, and strabismus.

Figure 14-15 shows skin and eye de ects that may occur in this syndrome. Note the atrophy on the orehead, the ocular de ect in the right eye, and the papillomatous changes on the chin. Papillomas may be present on the lips or in the axillae, periumbilical area, or perineum.

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Fig ure 14-16

Focal dermal hypoplasia (Goltz syndrome) Figure 14-16 illustrates the requent involvement o the perioral skin and teeth. Patients may present with hypodontia, oligodontia, or small teeth with dysplastic enamel.

Fig ure 14-17

Fig ure 14-18

Incontinentia pigmenti T is rare condition is characterized by linear and whorled lesions and a wide variety o systemic mani estations. Incontinentia pigmenti is inherited as an X-linked dominant trait linked to the NEMO gene and is seen almost exclusively in girls. T e cutaneous eruption is usually present at birth and evolves through three stages.

Lesions typical o the rst two stages, vesicular and verrucous, are seen in Figs. 14-17 and 14-18. T e vesicular phase o incontinentia pigmenti can be quite extensive and lasts or about 2 weeks. Recurrence o vesicular lesions during childhood has also been reported to occur.

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Se ction 1 4 . Ge node rm atos e s

Fig ure 14-19

Fig ure 14-20

Incontinentia pigmenti T e vesicular lesions in this disorder must be di erentiated rom herpes simplex and other causes o blistering in newborn in ants. A skin biopsy o en provides de nitive evidence o the diagnosis.

Vesicular and verrucous lesions ollowing the lines o Blaschko are shown in Fig. 14-20. T e disorder may also result in scarring alopecia o the scalp, dystrophic nails, and abnormalities o the teeth.

Fig ure 14-21

Fig ure 14-22

Incontinentia pigmenti A combination o vesicular and verrucous lesions are seen in this newborn girl.

T e verrucous phase lasts or about 6 weeks, although it may go on or many months or even or years in rare cases.

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Fig ure 14-23

Fig ure 14-24

Incontinentia pigmenti Over several months the raised areas f atten, and the patient develops whorled, or “marble-cake,” hyperpigmentation, as pictured in Figs. 14-23 to 14-25. In turn, the lesions o this third, hyperpigmented stage ade over a period o several years.

T e patients represented in Figs. 14-23 and 14-24 both show details o the marbled hyperpigmentation as well as remnants o the previous papular verrucous stage. A ourth, hypopigmented stage can develop in the second and third decade.

Fig ure 14-25

Fig ure 14-26

Incontinentia pigmenti T is illustration is a good representation o extensive dyschromia. Central nervous system and ocular abnormalities are the most serious aspects o this disease. Some patients will develop seizures, mental retardation, or spastic paralysis. Eye involvement may include the presence o a retrolental mass, retinal detachment, cataracts, and optic atrophy. Skeletal anomalies are sometimes seen.

Incontinentia pigmenti T e most common extracutaneous abnormality in incontinentia pigmenti involves teeth and occurs in about two-thirds o patients. T ere may be a marked delay in the eruption o deciduous teeth. Dental de ects such as partial or complete absence o teeth as well as conical teeth may be seen.

161

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-27

Wiskott-Aldrich syndrome T is is another severe immunologic de ect with X-linked recessive inheritance. T e classic symptoms o this disease, which occurs only in males, are thrombocytopenia, recurrent in ection, and a generalized eczematous or ex oliative dermatitis. Children with this disorder have impaired humoral and cell-mediated immunity, with de icient IgM and elevated IgA, and are at risk or sepsis and hemorrhage. Figure 14-27 shows the kind o petechiae that are evidence o the persistent thrombocytopenia.

Fig ure 14-28

Fig ure 14-29

Ataxia telangiectasia T is autosomal recessive disorder, caused by a de ect in the ATM gene, a ects the skin and the immunologic and central nervous systems. T e onset o the disease, during early childhood, is characterized by the combination o progressive cerebellar ataxia and telangiectasias. T e earliest site o telangiectasia is usually the bulbar conjunctiva, as pictured in Fig. 14-28. T ese vascular lesions also involve the neck, upper chest, ace, and, as illustrated in Fig. 14-29, the pinna o the ear.

Later cutaneous changes include blotchy hyper- and hypopigmentation, ca é-au-lait spots, hirsutism, a generalized eczematous dermatitis, and granulomatous skin lesions. T ere may be premature graying o the hair. T e immunologic abnormalities include decreased levels o IgA and IgE and de ective cell-mediated immunity. Children with ataxia telangiectasia su er rom recurrent sinopulmonary in ection and may die rom bronchiectasis and respiratory ailure. Lymphoreticular malignancies are an additional complication.

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Fig ure 14-30

Fig ure 14-31

Bloom syndrome T e principal cutaneous mani estations o this autosomal recessive syndrome are erythema and telangiectasias o the cheeks and photosensitivity. Ca é-au-lait spots and acanthosis nigricans may also be present. Children with Bloom syndrome are small at birth and have severe growth retardation throughout li e. T ey also have recurrent respiratory in ections and a strong tendency to develop malignancy. T is rare disease is due to chromosomal abnormalities and de ects in DNA repair. T e syndrome is caused by mutation in the gene designated BLM, traced to band 15q26.1.

Rothmund-T omson syndrome (Poikiloderma congenitale) T is rare autosomal recessive disorder is attributed to mutations o the RECQL4 helicase gene on chromosome 8q24. T e condition begins during in ancy with typically progressive skin changes. Erythema and edema o the acial skin are rapidly ollowed by the development o atrophy and telangiectasia. T e same process occurs on the buttocks and extremities. Cataracts develop in many patients, and these o en become apparent during in ancy or early childhood. Skeletal de ormities and short stature are other occasional eatures o this disease.

Fig ure 14-32

Fig ure 14-33

Cockayne syndrome T is is another rare autosomal recessive condition characterized by skin changes and dwar sm. During the second year o li e, patients develop a scaly photoeruption on the ace and upper neck. T is resolves with hyperpigmentation. T e scaling photodermatitis and typical bird-like acies o this syndrome are illustrated in Fig. 14-32.

In addition to dwar sm (Fig. 14-33), a ected individuals may demonstrate sensorineural dea ness, microcephaly, and severe mental retardation. Optic atrophy and retinal degeneration may also be present. Characteristic intracranial calci cations are a eature o Cockayne syndrome that may aid in con rmation o the diagnosis. T e disorder is due to a de ect in DNA repair.

163

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-34

Fig ure 14-35

Hypohidrotic ectodermal dysplasia T e ectodermal dysplasias are a wide variety o genetic disorders that may a ect development o the hair, nails, teeth, and sweat glands. In this most common orm, patients lack the ability to sweat and are at risk or developing hyperthermia in warm environmental conditions. T ere is sparse scalp and body hair and the hair may be, brittle, and slow-growing. Children may have absent teeth or teeth that are small and pointed.

T e acial eatures o hypohidrotic ectodermal dysplasia include thick lips, a prominent orehead, and a f attened nasal bridge. T ere is o en dark and thickened skin around the eyes, and children with this disorder are prone to atopic dermatitis. Most cases are caused by mutations in the EDA gene, which is inherited in an X-linked recessive pattern. Other orms are either autosomal dominant or autosomal recessive.

Fig ure 14-36

Fig ure 14-37

Clouston syndrome T is less common orm o ectodermal dysplasia is inherited in an autosomal dominant ashion and is due to a mutation in the GJB6 gene. Abnormal ngernails and toenails are a hallmark o this disorder. T e nails are white in early childhood, and later become thick and brittle.

T e nail dystrophy may be accompanied by severe hyperkeratosis o the palms and soles, as seen in Fig. 14-37. opical keratolytics are o some help to a ected individuals. Other eatures o this disorder are hyperpigmentation o the skin over the joints, and clubbing o the nails. Sweating is normal.

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Fig ure 14-38

Fig ure 14-39

Hay-Well syndrome (AEC) Most in ants with this autosomal dominant disorder caused by mutation in the TP63 gene are born with generalized erythematous and scaly skin. T ere may be signi cant erosions as noted on the scalp and buttocks in the in ant represented in Figs. 14-38 and 14-39. T e erosions can lead to scarring and hair loss.

Skin erosions may occur throughout childhood and at time into adulthood. Other common eatures include the presence o ankyloblepharon (small strands o skin between the eyelids) at birth, and cle lip and palate. Limb abnormalities such as syndactyly may be seen.

Fig ure 14-40

Fig ure 14-41

Hay-Well syndrome (AEC) Many patients with this syndrome have persistent problems with an erosive scalp dermatitis, with requent secondary in ection and excessive granulation tissue.

T e hair on other parts o the scalp tends to be wiry and coarse. Scarring and hypotrichosis is noted in the patient in Fig. 14-40. In Fig. 14-41 there are pustules scattered throughout the scalp.

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Fig ure 14-42

Fig ure 14-43

Pachyonychia congenita T is condition results rom mutations in the genes encoding epidermal keratinocyte keratins, and a ects nails, palms and soles, and the mucous membranes o the lips and mouth.

T e nail changes are distinctive in their discoloration, hardness, excessive growth, and attachment to hyperkeratotic nail beds. In Fig. 14-43, scleronychia and binding to the nail beds can be appreciated.

Fig ure 14-44

Fig ure 14-45

Pachyonychia congenita Figure 14-44 illustrates the severe hyperkeratotic process on the soles o the eet. T e palms may also be involved. T e plantar involvement can be particularly pain ul and inter ere with normal unction.

Patients with pachyonychia congenital also develop leukokeratosis o the tongue. Oral lesions occur in approximately 70% o patients, but do not evolve into malignancy.

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Fig ure 14-46

Fig ure 14-47

Dyskeratosis congenita T is rare congenital condition results rom mutations in at least 10 known genes and occurs in X-linked recessive, autosomal dominant, and autosomal recessive subtypes. It is characterized by atrophy and dyschromia o the skin, atrophic dysplasia o nails, and leukoplakia. Figure 14-46 illustrates the typical mottled or reti orm poikiloderma. T e ace, neck, and upper part o the body are characteristically involved.

Mucous membrane involvement is shown in Fig. 14-47. T ere are two lesions on the tongue that are gray and hypertrophic. T e buccal mucosa and the mucosa o the urethra and anus may also be a ected. Aside rom the cutaneous and mucosal e ects, abnormalities such as those seen in Fanconi syndrome may be associated with this condition.

Fig ure 14-48

Fig ure 14-49

Dyskeratosis congenita Fig. 14-48 shows hypoplastic nail changes that are characteristic. Note how di erent they are rom those o pachyonychia congenita (Figs. 14-42 and 14-43).

Details o the cutaneous changes on glabrous skin in and around the groin, similar to those that occur on the neck and ace, are shown in Fig. 14-49. Hyperhidrosis o palms and soles, conjunctivitis, esophageal strictures, intestinal diverticula, mental retardation, and anemia may be part o the syndrome. Malignant degeneration in the leukoplakia o the mucous membranes can occur.

167

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-50

Fig ure 14-51

Neurof bromatosis ype I T is autosomal dominant disorder includes a number o distinctive cutaneous ndings and a wide variety o neurologic mani estations. Figure 14-50 shows multiple ca é-au-lait macules. Solitary lesions o this type are common in normal individuals; most patients with neuro bromatosis have more than a single macule. T e presence o more than six lesions that are larger than 0.5 cm in diameter in prepubescent children and 1.5 cm in diameter in adults is considered one o the major diagnostic criteria or this disease.

In Fig. 14-51, there are numerous macules o hyperpigmentation in the axilla. Axillary reckling, also called Crowe sign, is a unique nding in neuro bromatosis. Pigmented hamartomas o the iris, termed Lisch nodules, are also present in almost all patients with this condition. Optic gliomas may also be seen. T e inheritance o neuro bromatosis is complicated by a highly variable range o expression among those a ected. A single amily may include some individuals with only cutaneous involvement and others with numerous neuro bromas or severe neurologic disease. Spontaneous mutations are also common.

Fig ure 14-52

Fig ure 14-53

Neurof bromatosis ype I T ese are more illustrations o the pigmentary anomalies and tumors in the skin o patients with neuro bromatosis. Figure 14-52 shows several ca é-au-lait spots and small tumors. Figure 14-53 shows a large ca é-au-lait spot on the le and a tumor on the right. Neuro bromas have variable consistency. T e so lesions can be pushed into the surrounding skin, a process called “buttonholing.” Neuro bromas usually begin to develop during puberty and may cause severe

cosmetic dis gurement. Pruritus o the skin that overlies the neuro broma is a common complaint, and the itching is aggravated by exertion or a warm environment. Malignant degeneration o a benign neuro broma can occur. In addition, patients with the disease are prone to developing neuro brosarcomas or malignant schwannomas de novo. Selective surgical removal o neuro bromas, in order to improve either appearance or unction, is easible.

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Fig ure 14-54

Fig ure 14-55

Neurof bromatosis ype I Figure 14-54 shows three ca é-au-lait spots and a small, so neuro broma. Intellectual handicap, and seizures are requent mani estations o classic, or von Recklinghausen, neuro bromatosis.

T e enlargement in Fig. 14-55 was ound to be due to a neuro broma in the palm and along the length o the digits. Although neuro bromatosis tends to be progressive, it is entirely unpredictable. T ere is no single aspect o the clinical course that allows the physician to oresee the evolution o other eatures.

Fig ure 14-56

Fig ure 14-57

Neurof bromatosis ype I Figure 14-56 illustrates several large ca é au lait spots, and an area o grayish mottled hyperpigmentation that probably overlies a plexi orm neuro broma. T ese subcutaneous tumors are pathognomonic or neuro bromatosis type I.

Plexi orm neuro bromas may sometimes be subtle, initially presenting only as a patch o hyperpigmentation and/or hypertrichosis. With time, they can grow quite large.

169

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-58

Fig ure 14-59

Neurof bromatosis type I Larger plexi orm neuro bromas are described as having a “bag o worms” consistency and may be cosmetically disabling.

Figure 14-59 shows a large plexi orm neuro broma with overlying darkening and thickening o the skin. Plexi orm neuro bromas may also present as rm nodules attached to the nerves. umors around nerves may cause pain, muscle weakness, or atrophy.

Fig ure 14-60

Fig ure 14-61

Neurof bromatosis type I Plexi orm neuro bromas can invade the orbit or eyelids, obscure the visual axis and cause amgblyopia. In the long term, in ltration o these anatomical structures is potentially vision threatening.

Neurof bromatosis type I (Lisch nodule) Lisch nodules are melanocytic hamartomas occurring on the iris. T ey can be seen on routine examination o the eye, but a slit lamp is required to distinguish these lesions rom nevi. Lisch nodules are brown and dome-shaped and measure up to 2 mm in diameter. T e majority o patients with NF1 develop this nding during childhood, and the nodules are present in the vast majority o adolescents and adults with the disorder. T e nodules do not cause any ophthalmologic complication.

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Fig ure 14-62

Fig ure 14-63

Multiple endocrine neoplasia type 2 T e presence o numerous small neuromas o the lips, tongue, and oral mucosa is a marker or a unique autosomal dominant condition. T is rare amilial syndrome, ormerly termed mucosal neuroma syndrome, is caused by mutations in the RET proto-oncogene, and carries a very high risk or malignancy. Eighty percent o a ected individuals will

develop medullary thyroid carcinoma. Pheochromocytoma may also occur. T e patient in Fig. 14-62 developed both tumors and had the mar anoid habitus that is requently seen in patients with the disorder. Note the small lesions in the lower eyelid in Fig. 14-63.

Fig ure 14-64

Fig ure 14-65

uberous sclerosis T is autosomal dominant disease with widely variable penetrance has its main e ects on the skin, central nervous system, eye, kidney, and heart. Figures 14-64 and 14-65 illustrate angio bromas, previously named adenoma sebaceum, one o the most common cutaneous mani estations. T e pink-to-red dome-shaped papules usually appear between the ages o 2 and 6, and, early in the clinical course, can be conused with acne. T e angio bromas may be symmetrically distributed over the entire ace but are usually most concentrated on the cheeks.

In some patients topical sirolimus may be an e ective treatment o angio bromas. T e extent o cutaneous involvement is not generally predictive o the severity o the systemic disease. Patients who are severely a ected su er rom seizure disorders and mental retardation. Other ndings o tuberous sclerosis include retinal and renal hamartomas, cerebral nodules and calci cations, and cardiac rhabdomyomas. T is disorder is due to mutations in TSC1 (hamartin gene) or TSC2, (tuberin gene).

171

Se ction 1 4 . Ge node rm atos e s

Fig ure 14-66

Fig ure 14-67

uberous sclerosis Figure 14-66 illustrates subungual and periungual bromas, also known as Koenen tumors. T ese rm lesions arise rom the nail beds, usually a er puberty. Periungual bromas may occur in both ngernails and toenails, and can be pain ul and dis guring.

Con etti-like hypopigmented macules may occur on the extremities o children with tuberous sclerosis. T is orm o hypopigmentation is signi cantly less common than larger hypopigmented macule (ash lea spot) illustrated in Fig. 14-68.

Fig ure 14-68

Fig ure 14-69

uberous sclerosis T is gure shows a hypopigmented macule, which sometimes takes the shape o an ash lea . Hypopigmented macules may occur in healthy in ants, but the appearance o several lesions should prompt a search or other mani estations in the patient and amily members. T ese spots are either present at birth or evolve during in ancy. Wood light examination may sometimes reveal hypopigmented macules whose presence is otherwise not obvious.

Shown in the center o Fig. 14-68, and in this photo (Fig. 14-69) is the shagreen patch. T e lesion, requently seen in children with tuberous sclerosis, is an area o cutaneous thickening with a pebbled sur ace. Histologically, this lesion is a orm o connective tissue nevus.

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Fig ure 14-70

Fig ure 14-71

uberous sclerosis Another cutaneous nding in tuberous sclerosis is the development o rm brous plaques that are located on the orehead, scalp, and cheeks. T ese lesions, which may be present at birth, are di erent rom angio bromas in that there is no vascular dilatation associated with the dermal brosis.

Buschke-Ollendor syndrome T is autosomal dominant syndrome is characterized by the presence o connective tissue nevi o the skin and a radiologic abnormality known as osteopoikilosis. T e connective tissue nevi are yellowish plaques that tend to appear be ore puberty and are present on the trunk, buttocks, and arms. Osteopoikilosis is an asymptomatic bony abnormality that presents as round opacities within the carpal and tarsal bones, the phalanges, the epiphyses and metaphyses o the long bones, and the pelvis.

Fig ure 14-72

Fig ure 14-73

Basal cell nevus syndrome Patients with this autosomal dominant syndrome caused by gene de ects in the PTCH1, PTCH2, and SUFU genes develop multiple basal cell carcinomas and cysts within the mandible. Additional ndings include hypertelorism, a variety o other skeletal abnormalities, and calci cation o the alx cerebri. Shown in Fig. 14-72 is an example o multiple small basaloid proli erations.

Most patients with basal cell nevus syndrome have more conventional basal cell carcinomas in the orm o pearly telangiectatic nodules, sometimes with ulceration, as is shown in Fig. 14-73. Basal cell carcinomas usually develop in these patients a er puberty, although they may occur in childhood.

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Se ction 1 4 . Ge node rm atos e s

Fig ure 14-74

Fig ure 14-75

Basal cell nevus syndrome Figure 14-74 shows the palmar pitting that appears during puberty in individuals with this syndrome. Rapidly enlarging basal cell carcinomas must be treated early on with the conventional surgical modalities in order to minimize dis gurement and avoid loss o unction. Patients with this syndrome are also at risk or medulloblastoma. T is grave complication tends to occur in early childhood.

Xeroderma pigmentosum T is rare autosomal recessive disease is caused by an inability to repair DNA that has been damaged by ultraviolet light. T ere are at least eight di erent molecular de ects. Freckling develops on sun-exposed skin at a very early age along with xerosis, scaling, and telangiectasia. Speckled hypoand hyperpigmentation are also typical.

Fig ure 14-76

Fig ure 14-77

Xeroderma pigmentosum Patients with this disease also tend to develop hyperkeratoses and changes consistent with actinic keratoses. In early childhood, patients with xeroderma pigmentosum begin to develop cutaneous malignancies: basal cell and squamous cell carcinomas and, less commonly, melanomas.

T e mortality rom this disease is caused by destructive local growth o tumors and by metastases. In addition, patients may su er rom severe neurologic dys unction, including mental retardation. Although no treatment is available, individuals with this disease bene t rom a li estyle that allows minimal exposure to sunlight.

SECTION

15 Ichthyoses and Disorders o Keratinization

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Fig ure 15-1

Fig ure 15-2

Ichthyosis vulgaris T is is the mildest and most common orm o ichthyosis, with an incidence in school-aged children as high as 1:250. It is inherited as an autosomal dominant trait and is present in a signi cant percentage o individuals with atopic dermatitis. It is not present at birth.

T e clinical appearance o this ichthyosis varies, depending on location. Figures 15-1 and 15-2 illustrate the ne, bran-like scaling on the upper chest and back. Children with ichthyosis vulgaris are likely to have increased skin markings on the palms and soles and a high incidence o keratosis pilaris (see Figs. 15-61 to 15-64).

Fig ure 15-3

Fig ure 15-4

Ichthyosis vulgaris On the anterior lower leg, there are o en larger, plate-like scales that resemble the skin o a sh (Figs. 15-3 and 15-4). Facial involvement is usually minimal, and exural areas are typically spared.

Ichthyosis vulgaris tends to worsen in winter when there is less sweating and lower humidity. reatment o ichthyosis vulgaris entails the use o emollients and creams and ointments containing urea, lactic acid, and other alpha-hydroxy acids. Excessive bathing and the use o alkaline soaps should be avoided. T e exacerbation that requently occurs in winter months can be lessened i a humidi er is used in the child’s room.

Se ction 1 5 . Ichthyos e s and Dis orde rs of Ke ratinization

177

Fig ure 15-5

Fig ure 15-6

Harlequin–type ichthyosis T e newborn in ant is covered with thick plates o scale that are o en described as resembling a coat o armor. A er birth, deep erythematous ssures orm between areas o scale. T ere is also severe acial dis gurement due to eclabium, ectropion, and edema o the conjunctiva. T e texture o the skin results in restriction o the respiratory movements o the chest and inter eres with normal eeding. T is orm o ichthyosis is due to a mutation on the ABCA12 gene.

Collodion baby T is is a descriptive term or the child who is born encased in a taut, parchment-like membrane, accompanied by ectropion and eclabium. T e outcome o this process is unpredictable. When the membrane is completely shed, the in ant may go on to develop one o several ichthyosis skin types. Lamellar ichthyosis and congenital ichthyosi orm erythroderma (pictured in Fig. 15-6) are the most common. A small percentage o in ants go on to have completely normal skin, a phenomenon called “sel -healing collodion baby.”

Fig ure 15-7

Fig ure 15-8

Collodion baby Collodion babies are at risk or complications. T ese include in ection, dehydration, body temperature instability, and pneumonia. Collodion babies should be placed in a high humidity environment, and monitored closely. Gradually, the membrane will come o on its own. T e child pictured in Fig. 15-7 also developed congenital ichthyosi orm erythroderma.

Figure 15-8 is an example o more severe orm o collodion baby, with marked ectropion. A er spontaneous shedding o the collodion membrane, this child went on to develop lamellar ichthyosis (Figs. 15-9 to 15-16).

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Fig ure 15-9

Fig ure 15-10

Lamellar ichthyosis Collodion babies, irrespective o the cause, are very o en born prematurely. In addition, they are at risk or cutaneous in ection, sepsis, pneumonia, and require care ul supportive therapy. T is in ant was ormerly a collodion baby and has gradually sloughed his membrane. When the baby was several months o age, he began to develop generalized hyperkeratosis and scaling. T is in ant has the chronic and severe autosomal recessive disease o lamellar ichthyosis.

T e child in Fig. 15-10 has severe acial involvement. Note the ectropion and tightness o the acial skin as a result o hyperkeratosis. Plate-like scales o the orehead are a particularly common eature.

Fig ure 15-11

Fig ure 15-12

Lamellar ichthyosis Figure 15-11 shows in detail the kind o ichthyosis that gradually becomes established a er the disappearance o the collodion membrane. Note the mosaic pattern o the scales and tendency o the edges o the scale to curl away rom the sur ace. T e scales are sometimes compared with armored plates. Blister ormation does not occur in this condition.

In severe cases, the entire skin sur ace, including the ace, is a ected by the hyperkeratotic, scaly, dyschromic dyskeratinization. Involvement tends to be most severe in exural areas, as in the axilla pictured in Fig. 15-12. opical therapy with the α-hydroxy acids, such as lactic acid, or with topical N-acetylcysteine is somewhat help ul in reducing the amount o scale and improving the appearance.

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179

Fig ure 15-13

Fig ure 15-14

Lamellar icthyosis (cont’d.) In this severe example, there is thick and dark scale involving the entire ace, as well as severe ectropion. For many children, eye involvement like this is the most dis guring aspect o the disease. In addition, scaliness o the scalp may be accompanied by partial hair loss.

T is patient has developed thick scale involving the entire skin sur ace. One orm o lamellar ichthyosis is associated with a de ciency o the enzyme keratinocyte transglutaminase.

Fig ure 15-15

Fig ure 15-16

Lamellar ichthyosis Figures 15-15 and 15-16 illustrate involvement o the palms and soles with thick hyperkeratosis and deep grooves.

In addition to thickening and ssuring o the palms and soles, the nails may be ridged or thickened, and there may be thick subungual hyperkeratosis.

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Fig ure 15-17

Fig ure 15-18

Recessive X-linked ichthyosis T is disorder is characterized by ichthyosis that begins at or shortly a er birth and persists through adult li e. T e “dirty” brown and tightly adherent scales are illustrated in Figs. 15-17 to 15-19. T e scaling tends to avor the trunk and the extensor sur aces o the extremities. T ere is relative sparing o the ace and exural areas.

T e palms and soles are also spared, and the hair and teeth are normal. Most patients note marked improvement during the summer months, probably related to improved skin hydration. Asymptomatic corneal opacities on the posterior membrane serve as an adult marker or this disease.

Fig ure 15-19

Fig ure 15-20

Recessive X-linked ichthyosis In addition, there is a signi cant incidence o cryptorchidism in individuals with this syndrome. T e locus or this rare genodermatosis is now known to a ect the S S (steroid sul atase) gene on the distal short arm o the X chromosome, and the disease is inherited in X-linked recessive ashion. T e underlying metabolic disorder is a de ciency in the enzyme steroid sul atase.

Figure 15-20 gives a closer view o the tightly adherent scales that are seen in this hereditary disorder.

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181

Fig ure 15-21

Fig ure 15-22

Epidermolytic ichthyosis ( ormerly epidermolytic hyperkeratosis) T e appearance o epidermolytic ichthyosis in a newborn is illustrated in Fig. 15-21. ypically, there are large areas o denuded skin, and sometimes there are intact blisters. T e di erentiation rom epidermolysis bullosa can usually be made by positive amily history, the presence o subtle areas o hyperkeratosis, and, most important, the characteristic skin biopsy. reatment in the newborn period should ocus on gentle handling to avoid new blister ormation, the maintenance o uid and electrolyte balance, and the prevention o bacterial superin ection.

Over time, the generalized blistering resolves, and widespread areas o thick hyperkeratosis and scale develop. In Fig. 15-22, there is a mixed picture; ocal erosions are present on the arms and abdomen, and there are areas o thick, discolored, urrowed hyperkeratosis. Note the predilection, which is not seen in ichthyosis vulgaris, or the antecubital ossae and intertriginous spaces.

Fig ure 15-23

Fig ure 15-24

Epidermolytic ichthyosis ( ormerly epidermolytic hyperkeratosis) T is condition was also previously called bullous congenital ichthyosi orm erythroderma. It is inherited in an autosomal dominant ashion. T e genetic de ect lies in mutations in genes encoding keratins 1 and 10. T e ormer is associated with the additional involvement o the palms and soles. Figures 15-23 and 15-24 show bullous ichthyosis in its most severe and generalized orm.

Patients with disease ace a very dif cult set o problems. Bacterial colonization o this thickened and urrowed skin may result in an extremely unpleasant body odor. T e oul smell, along with the dis gurement caused by the disease itsel , may inter ere enormously with social adaptation. Antibacterial soaps are o some help in reducing odor.

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Fig ure 15-25

Fig ure 15-26

Epidermolytic ichthyosis ( ormerly epidermolytic hyperkeratosis) Some children remain prone to mechanically induced blisters in areas o riction or trauma. reatment with keratolytics will o en induce pain ul super cial erosions, and the use o oral retinoids, with all o their side e ects, has met with little success.

Figure 15-26 illustrates both the darkening and thickening o the skin and the areas o adjacent super cial erosion. Fortunately, in many patients the disease tends to localize to smaller, usually exural, areas with advancing age.

Fig ure 15-27

Epidermolytic ichthyosis ( ormerly epidermolytic hyperkeratosis) Figure 15-27 illustrates the abdomen o a patient with epidermolytic ichthyosis. Note the dark, thickened scale with areas o desquamation that appear to have somewhat more normal skin than the surrounding areas that appear to have a slightly licheni ed appearance.

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183

Fig ure 15-28

Fig ure 15-29

Nonbullous congenital ichthyosi orm erythroderma T is rare autosomal recessive condition may present with a collodion membrane at birth. T e severity o the disease during in ancy and later childhood is extremely variable.

T e most typical appearance eatures widespread scaling and moderate to severe erythroderma. T e children with most severe involvement may experience dif culty with temperature regulation, and should be ollowed closely or evidence o secondary in ection.

Fig ure 15-30

Nonbullous congenital ichthyosi orm erythroderma Most cases are caused by mutations in the ALOXE3, ALOX12B, and NIPAL4 genes. Improvement sometimes occurs at puberty.

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Fig ure 15-31

Fig ure 15-32

Erythrokeratoderma variabilis T is is a rare autosomal dominant condition characterized by migratory areas o erythema and distinct plaques o hyperkeratosis. From an early beginning in in ancy, there occur gurate and rapidly (within days) shi ing areas o bright erythema on the ace, anterior trunk, buttocks, and extensor aspects o the limbs. T ese may be brought on by changes in environmental temperature. In addition, there are xed and localized hyperkeratotic plaques.

Progressive symmetric erythrokeratoderma T is rare disorder develops in early childhood. T e lesions are symmetrical large, sharply circumscribed, hyperkeratotic plaques with brownish or orange-red discoloration. T e most common locations are the extensor limbs, shoulders, buttocks and ngers. In about hal o the patients, palms and soles are involved.

Fig ure 15-33

Fig ure 15-34

Progressive symmetric erythrokeratoderma As illustrated in Fig. 15-33, acial involvement may also occur. In contrast to patients with erythrokeratoderma variabilis, these lesions are nonmigratory. T erapeutic options are limited and the use o oral retinoids may be considered.

T is essentially autosomal dominant disease tends to progress until adolescence, and then may improve spontaneously during adult li e.

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185

Fig ure 15-35

Fig ure 15-36

Netherton syndrome T is rare autosomal recessive syndrome is associated with mutations in the SPINK5 gene. It mani ests itsel in a unique ichthyosis and hair sha abnormality. At birth, these patients may have a generalized ichthyosi orm erythroderma, as seen in Fig. 15-35. Severe hypernatremic dehydration may occur.

A er the newborn period, children with Netherton syndrome may go on to develop a generalized skin rash that is similar in appearance to severe atopic dermatitis. Patients may su er rom itching and requent superin ection, and are also prone to develop numerous ood allergies.

Fig ure 15-37

Fig ure 15-38

Netherton syndrome T e most distinctive cutaneous eature is termed ichthyosis linearis circumf exa, a collection o circinate, erythematous, and hyperkeratotic lesions with a very characteristic double-edged scale along the margin (Fig. 15-37 and 15-38).

Patients with Netherton syndrome tend to have an atopic diathesis, with some combination o asthma, allergic rhinitis, and eczematous dermatitis. Finally, there is a tendency toward impaired immunity (elevated IgE and immature natural killer cells) and, in some cases, developmental delay.

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Fig ure 15-39

Fig ure 15-40

Netherton syndrome T e associated hair abnormality is seen in Fig. 15-39. Microscopic examination o these “bamboo hairs” (Fig. 15-40) most o en reveals a hair sha abnormality termed trichorrhexis invaginata, a ball-and-socket insertion o the distal hair sha into the proximal hair.

T ere may also be pili torti or trichorrhexis nodosa (Figs. 29-34 through Fig 29-37). T e hair may be absent at birth and the hair sha abnormality may sometimes be rst ound upon examination o eyebrow hair. T e hair disorder tends to correct itsel with the passage o time.

Fig ure 15-41

Fig ure 15-42

Palmoplantar keratoderma T ere is a long list o genodermatoses that includes thickening o the palms and soles as either the principal or an associated abnormality. T e most common among all o these is Unna-T ost palmoplantar keratoderma, which is caused by mutations in the KRT1 gene. Well-demarcated hyperkeratosis is noted on the palms and soles with a red

band noted at the periphery o the area and is sharply demarcated. T e autosomal dominant inheritance o this condition is illustrated by the mother–son involvement in Fig. 15-41 and 15-42. reatment consists o the use o keratolytic agents and various methods o scraping and paring to remove thickened skin.

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187

Fig ure 15-43

Fig ure 15-44

Conradi-Hünermann syndrome T is is a rare genetic disorder with multiple systemic e ects. A ected in ants have generalized redness and scaling (ichthyosi orm erythroderma). As illustrated in Figs. 15-43 and 15-44, the lesions occur in a whorled or linear, blotchy pattern. Older children and young adults develop ollicular atrophoderma, and sometimes dark and light patches o skin and patchy scarring alopecia.

Conradi-Hünermann syndrome is a orm o chondrodysplasia punctata, a group o genetic disorders with shortening o bones, and stippled epiphyses.. T is disorder is also associated with cataracts (these may be present at birth or develop a er), short stature, and a characteristic acial appearance. Conradi-Hünermann syndrome is caused by mutations o the emopamil-binding protein (EBP) gene and is inherited as an X-linked dominant trait. It occurs almost exclusively in girls.

Fig ure 15-45

Fig ure 15-46

Sjögren-Larsson syndrome T is is a rare autosomal recessive genodermatosis that is characterized by spasticity, mental retardation, and congenital ichthyosis. A combination o scaling and erythroderma is seen in the newborn. Over time, the ichthyosis tends to localize to the lower abdomen (Fig. 15-45) and exural areas (Fig. 15-46). T ere may be mild involvement o the palms and soles.

T e combination o spasticity and psychomotor developmental delay is o en quite severe and may be accompanied by a seizure disorder. A speci c retinal lesion, the so-called glistening dots, is present in almost all patients with Sjögren-Larsson syndrome a er 1 year o age. T is syndrome is due to the de cient activity o atty aldehyde dehydrogenase (FALDH).

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Fig ure 15-47

Fig ure 15-48

Linear epidermal nevus T ese lesions consist o verrucous, hyperkeratotic papules that are closely grouped in a linear array. Epidermal nevi are o en present at birth but may arise during the rst year o li e, and occasionally later. T ey may occur on the head and neck, trunk, or extremities.

Figure 15-48 illustrates an epidermal nevus on the abdomen. T e lesion had been present since birth and there were no associated symptoms. T e whorled appearance o the lesion is due to the act that epidermal nevi characteristically ollow the lines o Blaschko.

Fig ure 15-49

Fig ure 15-50

Linear epidermal nevus T is linear lesion, present rom the time o birth, had gradually become thicker over the course o several years. T e bene t o surgical excision must be weighed against the risk o a scar at the site o surgery.

Figure 15-50 shows an extensive epidermal nevus on the ace and neck, again ollowing the lines o Blaschko. Epidermal nevi have no malignant potential, except or the extremely rare development o basal cell carcinoma in a preexisting lesion. Surgical treatment is required only when indicated or cosmetic considerations.

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189

Fig ure 15-51

Fig ure 15-52

Epidermal nevus syndrome Figures 15-51 and 15-52 show patients with widespread epidermal nevi. Some patients with this phenotype will be diagnosed with epidermal nevus syndrome, which is a group o congenital disorders with a variety o systemic eatures. De ects in neural crest lead to mal ormations in skeletal, cardiovascular, ocular, and endocrine systems, and may also result in lipomas. T e most requent mal ormation in the brain is hemimegalencephaly.

Epidermal nevus syndrome results rom genetic mosaicism with a lethal autosomal dominant gene, and a number o speci c mutations have been identi ed. Skeletal abnormalities seen in the epidermal nevus syndrome include limb hypertrophy, bone cysts, and incomplete ormation o certain bones. A higher than normal incidence o malignancy, particularly Wilms tumor, has also been associated with this disorder.

Fig ure 15-53

Fig ure 15-54

Inf ammatory linear verrucous epidermal nevus (ILVEN) his linear array o pruritic papules arises most o ten during childhood. he lesions are erythematous and scaling and are usually localized to the lower extremities or perineum. Sometimes this entity may be dif cult to distinguish rom psoriasis.

T e persistence o in ammation in one anatomic area and the absence o psoriatic lesions elsewhere avor the diagnosis o ILVEN. Histologically, it may resemble psoriasis or nonspeci c chronic dermatitis.

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Fig ure 15-55

Fig ure 15-56

Darier disease (keratosis ollicularis) T is autosomal dominant disorder usually begins during mid-childhood. It is caused by a mutation in the gene ATP2A2. T e primary lesion is a small, crusted papule; in some areas, these coalesce to orm hyperkeratotic, greasy plaques. T e lesions tend to avor the so-called seborrheic areas, and the upper back and chest, as shown in Figs. 15-55 and 15-56, are the most common locations. Small, warty papules on the dorsa o the hands are a common nding.

T e lesions tend to worsen in the summer; both humidity and ultraviolet light have a negative e ect on the disease process. Nail involvement is characteristic o Darier disease and may include red or white longitudinal streaks, thinning or thickening o the nail plate, and subungual hyperkeratosis.

Fig ure 15-57

Fig ure 15-58

Darier disease (keratosis ollicularis) In more severe cases, there is extensive scalp and acial involvement, and there are moist, erythematous vegetating plaques in the exures, as illustrated in Fig. 15-57. T e severity o Darier disease varies greatly among members o the same a ected amily. T e use o synthetic retinoids in the most severe cases must be balanced against the problems o long-term toxicity.

Acrokeratosis verruci ormis T is autosomal dominant disorder is characterized by numerous at-topped or verrucous hyperkeratotic papules that are usually localized to the dorsa o the hands and eet. T ey tend to recur a er surgical removal. Histologically, there are distinctive elevations o the epidermis that resemble church spires. It should be noted that patients with Darier disease (see Figs. 15-55 to 15-57) may have identical acral lesions, and this disorder is also associated with de ects in the ATP2A2 gene.

Se ction 1 5 . Ichthyos e s and Dis orde rs of Ke ratinization

191

Fig ure 15-59

Fig ure 15-60

KID syndrome T e keratitis-ichthyosis-dea ness (KID) syndrome is a multisystem disorder that, in its most severe orms, may be disabling. Cutaneous changes consist o thick keratoderma o the palms and soles, keratotic plaques on the ace and extremities, and a di use ichthyosis with ollicular accentuation. KID syndrome is caused by mutations in the GJB2 gene, which codes or the protein connexin 26.

Abnormal keratinization may be present at birth. Alopecia, nail dystrophy, and hypoplastic teeth are all part o this disorder. T e hearing impairment in this disease is progressive and due to neurosensory dea ness. Keratitis begins during childhood and is accompanied by neovascularization. Visual loss may be severe. Recurrent and atypical cutaneous in ections may also be seen in KID syndrome.

Fig ure 15-61

Fig ure 15-62

Keratosis pilaris T is is an extremely common asymptomatic condition that is o en amilial and is sometimes associated with atopic dermatitis or ichthyosis vulgaris. Pictured in Figs. 15-61 and 15-62 are typical lesions in the two most common areas o involvement: the lateral aspect o the upper arms and the anterior thighs. T e lesions are ne keratotic papules;

each represents a plug in the upper part o a hair ollicle. T e appearance o the involved areas is o en likened to chicken skin or goose esh; the sensation on palpation is a grater-like roughness. Keratosis pilaris tends to worsen during the winter months and abates somewhat a er puberty. Keratolytics and emollients are somewhat help ul.

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Fig ure 15-63

Fig ure 15-64

Keratosis pilaris Children with keratosis pilaris sometimes develop the condition’s typical ollicular papules on the cheeks. When this occurs, there may also be erythema associated with the roughness. T is condition is sometimes termed keratosis pilaris rubra aciei. Again, mild keratolytics are sometimes help ul.

Keratosis pilaris rubra aciei T is eruption is sometimes conused with acne, in which comedones are ound along with papules, pustules, and sometimes cysts. T e prognosis o acial keratosis pilaris is good, although this condition may persist on the extremities.

SECTION

16 Urticarial, Purpuric, and Vascular Reactions

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Fig ure 16-1

Fig ure 16-2

Urticaria A wheal is an edematous papule that may enlarge to orm a pink, sharply circumscribed, elevated plaque. T e typical lesions o urticaria, pictured in Figs. 16-1 and 16-2, have a suggestion o central clearing.

By de nition, the individual lesions o urticaria evolve quickly and resolve within 24 to 48 hours. T ey are usually accompanied by severe pruritus. Urticaria is an extremely common disorder, and the etiology o en remains unknown. In most children, the problem resolves spontaneously over time.

Fig ure 16-3

Fig ure 16-4

Urticaria Larger, more geographic lesions are pictured in Figs. 16-3 and 16-4. In the cases where a cause is established, the most common etiologies o urticaria are medications, oods (eg, nuts, strawberries, shell sh, and other sea oods), and viral and bacterial in ections.

Autoimmune disease, such as autoimmune thyroiditis, and malignancy are extremely rare causes. In the child with chronic urticaria, it is o en di cult or impossible to identi y a single cause. In these patients, one attempts to control the development o new lesions with a daily schedule o nonsedating antihistamines.

Se ction 1 6 . Urticarial, Purpuric, and Vas cular Re actions

195

Fig ure 16-5

Fig ure 16-6

Physical urticarias T ere are urticarias in which stroking, pressure, cold, heat, or sun exposure are causative. Figure 16-5 shows a wheal produced by stroking the skin with a degree o orce that would ordinarily cause nothing more than transient erythema. T e phenomenon, called dermographism, is present in a small percentage o normal individuals.

Figure 16-6 shows a large wheal produced by resting an ice cube on the orearm. Cold-induced urticaria may be acquired or inherited. In the most common, acquired orm, patients develop lesions shortly a er ingesting cold oods or liquids or shortly a er exposure to a drop in environmental temperature. Patients with this orm o sensitivity are at risk or laryngeal edema or circulatory collapse as a result o signi cant cold exposure. Antihistamines or doxepin are o some help in preventing attacks. In a very rare syndrome, contact o the skin with water, without respect to its temperature, produces wheals (aquagenic urticaria).

Fig ure 16-7

Fig ure 16-8

Erythema multiforme T is disorder is termed multi orme because the morphology o its lesions is so variable. T e primary lesion is most o en an erythematous macule that evolves into a papule. Early in the course, these lesions may easily be mistaken or urticaria.

As the lesions enlarge, they orm round or irregularly shaped plaques. T e central area may blister or become dusky in color; this change represents the necrosis o keratinocytes in areas o active involvement.

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Fig ure 16-9

Fig ure 16-10

Erythema multiforme Figure 16-9 demonstrates the targetlike quality o the variably sized and shaped plaques. Note the redness and edema at the border and the duskier appearance at the center. Mucosal lesions are not uncommon. Erythema multiorme is a sel -healing disease, with an average duration o about 2 weeks.

he dorsum o the hand is a particularly common location. Herpes simplex in ection is by ar the most common etiologic agent. In some patients, requent recurrences o herpes simplex and erythema multi orme require the use o prophylactic acyclovir or valacyclovir or extended periods o time. A wide variety o drugs, most commonly the sul onamides, may also cause this syndrome.

Fig ure 16-11

Fig ure 16-12

Erythema multiforme Erythema multi orme tends to be acral in distribution. Figure 16-11 illustrates both bulla ormation and the crusting o bullae that can occur during the course o the disease.

T is plaque-like lesion on the elbow illustrates the edematous border and the dusky center, a result o the necrosis o keratinocytes. Erythema multi orme must be distinguished rom urticaria multi orme (Figs. 16-21 to 16-23, 21-4,), which has some similar clinical eatures.

Se ction 1 6 . Urticarial, Purpuric, and Vas cular Re actions

197

Fig ure 16-13

Fig ure 16-14

Stevens-Johnson syndrome/toxic epidermal necrolysis Drugs are the major etiologic actor in the development o this severe disorder. T e most common agents are sul onamides, anticonvulsants, and NSAIDs. Mycoplasma pneumoniae is the most commonly associated in ectious agent. T e cutaneous lesions include xed erythematous macules, target lesions, and bullae.

T ere may be progression to widespread erythema and denudation, leaving underlying erosions. Frozen section processing o a biopsy specimen o toxic epidermal necrolysis allows or rapid diagnosis. In patients with erosions and blisters, one sees necrotic keratinocytes, severe degeneration o the basal layer, and a subepidermal separation.

Fig ure 16-15

Fig ure 16-16

Stevens-Johnson syndrome/toxic epidermal necrolysis he oral cavity is almost always involved, with bullae, ulcerations, and crusting most commonly presenting on the lips, buccal mucosa, and palate. racheal and bronchial involvement may result in breathing di iculty. Additionally, patients with Mycoplasma-induced Stevens-Johnson syndrome may have mucositis with minimal or absent skin lesions.

Ocular involvement requently occurs in this syndrome, signi cantly af ecting the bulbar conjunctiva. Long-term consequences include corneal damage and scarring, which may lead to permanent visual impairment.

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Fig ure 16-17

Fig ure 16-18

Stevens-Johnson syndrome/toxic epidermal necrolysis Fever, headache, cough, and malaise are requent eatures o this disorder. In addition to the lips (pictured in Fig. 16-17) and oral mucous membranes, esophageal involvement may lead to severe dysphagia and di culty eating and drinking. Involvement o the vulva and vagina in girls, and glans penis in boys may cause dysuria and urinary retention.

In Figs. 16-17 to 16-20, note the extensive involvement with large and small bullous lesions. T e mortality rate in this disease is signi cant; most deaths are related to superin ection and sepsis.

Fig ure 16-19

Fig ure 16-20

Stevens-Johnson syndrome/toxic epidermal necrolysis Patients with toxic epidermal necrolysis bene t enormously rom intensive medical care, in a burn unit when possible. Meticulous attention to the care o blistered and denuded skin and mucous membranes, along with monitoring or electrolyte imbalance, dehydration, and in ection signi cantly improves the possibility o survival.

Both systemic steroids and IVIG have been advocated or the treatment o Stevens-Johnson syndrome/toxic epidermal necrolysis. T ere is mixed evidence or both o these treatments and their use remains controversial.

Se ction 1 6 . Urticarial, Purpuric, and Vas cular Re actions

199

Fig ure 16-21

Fig ure 16-22

Urticaria multiforme T is condition, also known as acute annular urticaria, is a benign and airly common hypersensitivity reaction that presents with urticarial plaques and annular or arcuate urticarial lesions, along with acral edema.

Because the lesions may have an ecchymotic dusky center, this disorder is o ten misdiagnosed as erythema multi orme. Children with urticaria multi orme may have recently had a viral or bacterial in ection.

Fig ure 16-23

Fig ure 16-24

Urticaria multiforme Urticaria multi orme has also been linked to treatment with antibiotics, such as amoxicillin, cephalosporins, and macrolides. reatment may consist o combination therapy with an H1-antihistamine and an H2-antihistamine.

Sweet syndrome (acute febrile neutrophilic dermatosis) T is syndrome is characterized by pain ul, raised, erythematous plaques and nodules. T e cutaneous eruption is accompanied by spiking evers and a neutrophilic leukocytosis. Skin biopsy reveals a widespread in ltrate o polymorphonuclear leukocytes throughout the dermis. Sweet syndrome is o en associated with either malignancy or antecedent in ection. Systemic corticosteroids are the treatment o choice.

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Fig ure 16-25

Fig ure 16-26

Erythema annulare centrifugum T is is an erythematous and edematous lesion that gradually enlarges to orm annular, polycyclic, and gyrate shapes. T e lesions tend to resolve spontaneously but may reappear. Erythema annulare centri ugum has been attributed to a wide variety o causes.

In some cases, it is temporally related to the development o malignancy and resolves with treatment or removal o the tumor. In other patients, the disease has been related to super cial ungal in ections and a wide variety o viral and bacterial diseases.

Fig ure 16-27

Fig ure 16-28

Erythema elevatum diutinum T is is a rare skin disease that is characterized by purple or yellowish papules, nodules, and plaques that tend to cluster on the hands, eet, and extensor suraces o the extremities. T e skin overlying joints is a avored location. T e distribution is usually symmetrical, and the disease tends to be chronic. Lesions o erythema elevatum diutinum

may be asymptomatic but in some cases are pain ul. Figure 16-27 shows typical nodules and plaques on the elbow, and yellowish papules on the nger are seen in Fig. 16-28. T e etiology o this chronic leukocytoclastic vasculitis remains unknown, but an immune complex etiology has been suggested. Dapsone is one mode o treatment.

Se ction 1 6 . Urticarial, Purpuric, and Vas cular Re actions

201

Fig ure 16-29

Fig ure 16-30

Progressive pigmented purpura (Schamberg disease) T is disorder is due to a mild orm o capillaritis, leading to extravasation o red cells and the deposition o hemosiderin. Af ected children have numerous discrete patches o petechiae.

T e most common location is the lower extremities. Over time, the individual lesions evolve into typical brown-orange macules containing “cayenne pepper” spots as shown in Fig. 16-30.

Fig ure 16-31

Fig ure 16-32

Progressive pigmented purpura (Schamberg disease) Figure 16-31 illustrates a severe case o pigmented purpura, involving both lower extremities. Although less common, pigmented purpura in a segmental distribution on one extremity has been reported to occur.

Lichen aureus is a localized orm more commonly seen in children and young adults consisting o a solitary or localized group o lesions that is more requently seen on the leg although any part o the body may be involved. Figure 16-32 shows lesions with a more golden color; only a ew petechiae are noted.

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Fig ure 16-33

Fig ure 16-34

Acute hemorrhagic edema of infancy his striking disorder occurs in in ants between 4 months and 2 years o age. he sharply circumscribed lesions avor the extremities and are edematous, ecchymotic, and purpuric. Edema o the ears and eyelids may also be noted.

Biopsy o an active lesion reveals an intense leukocytoclastic vasculitis. However, in contrast to Henoch-Schönlein purpura, systemic symptoms are very rare. T ere may be several outbreaks o new lesions, but the entire process generally resolves in approximately 2 weeks.

Fig ure 16-35

Fig ure 16-36

Traumatic purpura Figure 16-35 shows hemorrhage into the skin due to a di cult passage through the birth canal. In this gure, one sees edema and ecchymosis that resulted rom molding o the head in a prolonged spontaneous delivery.

T roughout li e, hard knocks in the physical sense are the lot o us all. Rupture o capillaries is thus exceedingly common, and more extensive rupture o arterioles, venules, arteries, or veins is common enough. Petechiae, ecchymoses, vibices, and hematomas are banal, nearly everyday events. T e black eye and contusions are o everyone’s experience. Illustrated in Fig. 16-36 is a traumatic ecchymosis that may well represent a hickey (“passion purpura”). (caption by Morris Leider, f rst edition o Color Atlas o Pediatric Dermatology, 1975).

Se ction 1 6 . Urticarial, Purpuric, and Vas cular Re actions

203

Fig ure 16-37

Fig ure 16-38

Henoch-Schönlein purpura Palpable purpura is part o a syndrome that is also marked by attacks o arthralgia, abdominal pain, and hematuria. he entire complex o visible cutaneous purpura, arthralgia, and visceral signs and symptoms results rom a widespread IgA-related vasculitis. In addition to purpura, the skin may show edematous plaques, vesicles, and even necrosis. T e cause o this condition is not known.

Abdominal pain occurs in up to 65 percent o cases. Usually the pain is colicky, and it can be associated with vomiting and hematemesis. T ere may also be gross or occult blood in the stools. In unusual cases, there is intussusception, hemorrhage and shock.

Fig ure 16-39

Fig ure 16-40

Henoch-Schönlein purpura T e most serious consequence o this disease is renal disease. Patients who develop kidney involvement may do so months a er the onset o cutaneous purpura. T e most common mani estation o renal disease is hematuria. Proteinuria and hematuria may rarely progress to renal insu ciency.

Figure 16-40 is a good representation o both the purpuric elements and the edema that may appear in the Henoch-Schönlein syndrome. Rare mani estations o this disorder are pulmonary hemorrhage and central nervous system involvement with seizures and behavioral changes. Scrotal swelling and testicular torsion has also been reported.

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Fig ure 16-41

Fig ure 16-42

Purpura fulminans T is condition is a rare and exceedingly serious consequence o certain acute in ectious diseases. Scarlet ever o bygone days, meningococcal meningitis, severe varicella, and congenital protein C de ciency have been known to be complicated by ulminating purpura that went on to gangrene, extreme toxicity, shock, and death.

T is is an additional example o purpura ulminans due to meningococcemia. T e cause o the condition is a necrotizing vasculitis associated with de ects o clotting (disseminated intravascular coagulation).

Fig ure 16-43

Fig ure 16-44

Aphthous stomatitis T is very common condition is characterized by recurrent episodes o pain ul ulceration on the lip, tongue, or buccal sur aces. T e individual lesions quickly evolve rom erythematous macules to papules and then to yellowish ulcerations with a surrounding pink or red halo.

T e etiology is not known, and the ulcers heal in 7 to 10 days without scarring. Whereas a solitary lesion is the cause o only temporary, minor discom ort, there are some individuals who develop deeper and larger ulcers or numerous erosions. For them, aphthous stomatitis can be a severe problem.

Se ction 1 6 . Urticarial, Purpuric, and Vas cular Re actions

205

Fig ure 16-45

Fig ure 16-46

Behçet syndrome T is rare disease is characterized by a classic triad o recurrent ulcerations o the oral mucosa, genital ulcers, and eye involvement. T e oral ulcers, pictured in Figs. 16-45 and 16-46, tend to be larger, more numerous, and more requently recurrent than those o simple aphthous stomatitis. Genital lesions occur on the penis and scrotum in males, and on the vulva in emales. T ese requently recurrent ulcers tend to heal with scarring. T e various orms o eye involvement include uveitis and keratoconjunctivitis and may eventuate in blindness.

Other cutaneous lesions may be widespread and include papules, vesicles, abscesses, and lesions o erythema nodosum. In addition to the eyes and skin, a number o other organ systems may become involved. Severe chronic arthritis and thrombophlebitis are common occurrences. Gastrointestinal disease ranges rom mild abdominal discom ort to chronic diarrhea and an ulcerative colitis-like illness.

Fig ure 16-47

Behçet syndrome ypical vaginal lesions are pictured in Fig. 16-47. Neurologic mani estations, including recurrent meningoencephalitis and brain stem lesions, are o en the most serious and can be li e threatening. Diagnosis o Behçet syndrome is based on the presence o the typical clinical ndings. T e disease is di cult to treat, but colchicine, immunosuppressive drugs, and corticosteroids are the most requently used therapies. umor necrosis actor inhibitors also have a varying degree o success.

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Fig ure 16-48

Fig ure 16-49

Erythema ab igne T is disorder results rom prolonged and repeated exposure to in rared radiation. Historically, and be ore the advent o central heating, erythema ab igne was seen on the legs o individuals who sat or stood in ront o heating devices. In this era, more common causes are hot water bottles and heating pads. T e patient in Fig. 16-48 used a heating pad or relie rom menstrual cramps; the patient in Fig. 16-49 applied heat to his thigh a er an injury.

Erythema ab igne on the anterior thighs may be caused by the warmth rom a laptop computer. ypical lesions are characterized by reticular erythema and brownish pigmentation. Epidermal atrophy and subepidermal blisters may also occur.

SECTION

17 Bullous, Pustular, and Ulcerating Diseases

208

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Fig ure 17-1

Fig ure 17-2

Pemphigus vulgaris Pemphigus vulgaris is a rare autoimmune, bullous disease that occasionally occurs during childhood. T e disease a ects both the skin and mucous membranes and can be li e threatening. T e typical lesions o pemphigus vulgaris are pictured in Fig. 17-1. Erosions o the lips, gums, tongue, and palate, as pictured here, are a common presenting symptom and may be misdiagnosed early in the course o the disease. T e di culty in chewing and swallowing that may occur can become a signi cant complication.

Cutaneous lesions consist o accid weeping blisters that quickly erode to leave large denuded areas o skin. Nikolsky sign, the extension o blistering by lateral nger pressure, is seen in the presence o widespread disease. Figure 17-2 shows the kind o crusting that develops as the blisters open. Antibodies to desmoglein 1 are associated with skin lesions and antibodies to desmoglein 3 are associated with oral lesions.

Fig ure 17-3

Fig ure 17-4

Pemphigus vulgaris T e blisters o pemphigus vulgaris may arise on an erythematous base, or on normal-appearing skin, as pictured in Fig. 17-3. A variety o modalities have been employed in the treatment o this disease. T e patient who is seriously ill requires hospitalization.

Figure 17-4 illustrates both intact blisters and super cial erosions. For most patients, the most rapidly e ective treatment remains high-dose systemic steroids. Patients undergoing this orm o therapy are at risk or in ection and must be ollowed with extreme care. Rituximab, a monoclonal antibody against the B cell sur ace protein CD20, is the most promising new treatment or this disease. Immunosuppressive agents such as azathioprine, mycophenolate mo etil, intravenous immunoglobulin, and plasmapheresis are use ul therapies.

Se ction 1 7 . Bullous , Pus tular, and Ulce rating Dis e as e s

209

Fig ure 17-5

Fig ure 17-6

Pemphigus vegetans When the cutaneous changes o pemphigus take place in intertriginous spaces, clear blistering is not evident. Rather, one sees boggy in ammation. T e essential histologic process is again epidermal acantholysis, but blister roo s part almost at once and secondary in ection is inevitable. T is gure is a good representation o the kind o clinical appearance that develops in pemphigus vegetans. Lesions on other parts o the body take the orm o pemphigus vulgaris.

Familial benign chronic pemphigus (Hailey-Hailey disease) T is blistering disease is inherited in autosomal dominant ashion. Onset tends to occur during late adolescence. Patients with this disorder have a pruritic vesicular eruption in intertriginous areas that is worse during the summer months. Intact bullae may be absent, and o en there is only an erosive and crusted intertrigo in the axillae, in the groin, and on the neck. Control o this condition is best achieved by the avoidance o the causative actor, such as heat or riction, and the treatment o superin ection when it occurs.

Fig ure 17-7

Fig ure 17-8

Subcorneal pustulosis (Sneddon-Wilkinson disease) T is condition is most common during adulthood, but it does occur in children. Characteristically, crops o vesicles and pustules spring up and evolve into areas o super cial crusts and scales. As the lesions coalesce, they orm the type o arcuate plaque that is illustrated in Fig. 17-7. T e most common locations are the axillae, groin, and exures o the upper and lower extremities. T e lesions are o en pruritic, and, especially in childhood, there may be ever and an elevated white blood count.

T e etiology o this condition is completely unknown. Diagnosis is best con rmed by classic histologic appearance, which consists o collections o neutrophils just beneath the stratum corneum. T e disease process is essentially benign, but dapsone is an e ective therapy or the patient with requent or severe recurrences.

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Fig ure 17-9

Fig ure 17-10

Pemphigus foliaceus T is orm o pemphigus is less severe than pemphigus vulgaris because blister ormation occurs higher in the epidermis. As a result, there is less compromise o vital cutaneous unctions. In most areas o the world, pemphigus oliaceus is extremely unusual in children. In rural areas o Brazil, there is an endemic orm o pemphigus oliaceus, termed fogo selvagem, which a ects individuals o all ages.

Pictured in Figs. 17-9 and 17-10 are the typical lesions o pemphigus oliaceus. T e disease o en begins in the scalp with areas o erythema and scaling. As it progresses to involve the trunk and extremities, there evolve numerous crusting and erythematous plaques. Severe scaling is common, but usually there is no blister ormation and no involvement o the oral cavity. reatment consists o topical or systemic steroids, depending on the severity o the disease.

Fig ure 17-11

Fig ure 17-12

Pemphigus foliaceus Lesions in the perioral area, as seen in Fig. 17-11, are quite common. Oral mucosal involvement is less common, in contrast to pemphigus vulgaris. O en, patients with pemphigus oliaceus are initially thought to have recurrent impetigo, although the in ection does not totally clear with appropriate antibiotics. It is important to realize that secondary in ection o en occurs in these patients, and this should be considered when patients have are-ups or the disease is dif cult to control.

Figure 17-12 shows annular lesions that are seen in childhood orms o pemphigus oliaceus. Direct immuno uorescence is important in making the diagnosis. In addition, antibodies to desmoglein, an epidermal desmosomal component, are ound.

Se ction 1 7 . Bullous , Pus tular, and Ulce rating Dis e as e s

211

Fig ure 17-13

Fig ure 17-14

Bullous pemphigoid T is autoimmune blistering disorder is due to antibodies to various components o the basement membrane. It is extremely rare during childhood. ense blisters can arise on either erythematous or normal skin and may be pruritic. Lesions tend to cluster and may be preceded by the development o urticarial plaques. Sites o predilection include the ace, groin, and inner thighs.

Palm and sole involvement is sometimes seen in children, and mouth lesions are extremely unusual. Blisters generally heal without scarring but may leave temporary hyper- or hypopigmentation. T e prognosis is generally good, and initial treatment consists o topical and/or systemic corticosteroids.

Fig ure 17-15

Vulvar pemphigoid Patients with bullous pemphigoid may develop both genital and oral lesions. Rarely, in young girls, pemphigoid is limited to the vulva and may result in scarring. Localized vulvar pemphigoid must be distinguished rom lichen sclerosus et atrophicus (Figs. 20-12 and 20-13), which can also result in chronic blistering and pain in this anatomic location.

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Fig ure 17-16

Fig ure 17-17

Epidermolysis bullosa simplex (EBS) T e most common types o epidermolysis bullosa simplex (EBS) are autosomal dominant and the lesions are localized. Other subtypes o EBS, including autosomal recessive orms, have now been characterized. T e blisters in EBS are in the epidermis, and can be either suprabasal or basal. Multiple genetic mutations have been identi ed; most orms o EBS are associated with mutations in genes coding or keratins 5 and 14.

Epidermolysis bullosa simplex begins at birth or in early in ancy, and throughout li e the site o blistering corresponds to areas o riction or other trauma. T e localized orm o EBS, previously known as the Weber-Cockayne type (Fig. 17-17), is associated with onset in early childhood and with blisters orming on the hands and eet. Milia and scarring are rarely associated with this orm, and hyperkeratosis o the palms and soles may develop in adulthood.

Fig ure 17-18

Fig ure 17-19

Epidermolysis bullosa simplex (EBS) During in ancy, blisters occur on the neck, lower extremities, and hands and eet. In the child who is old enough to wear shoes, the dorsa o the toes are a common site o bulla ormation. In this condition, cleavage occurs through the basal cell layer o the epidermis, and the dermoepidermal junction is otherwise undisturbed. T e lesions are super cial, but sometimes are pain ul and cause dif culty in walking. In children with this disorder, mucous membrane involvement is minimal and usually does not produce serious dif culty. T ere may be some nail dystrophy, but this also heals with the cutaneous process.

Epidermolysis bullosa simplex (Dowling-Meara) T is disorder is characterized by grouped, herpeti orm blistering that generally occurs on the trunk but is also seen on the ace and extremities. Blistering in in ancy is o en severe and extensive, and can be atal. A er a ew months, in ants develop requent blistering o the palms and soles, along with periungual lesions. T e blistering can lead to milia, scarring, dystrophic nails, and a di use palmoplantar keratoderma. Blistering tends to improve with age.

Se ction 1 7 . Bullous , Pus tular, and Ulce rating Dis e as e s

213

Fig ure 17-20

Epidermolysis bullosa, junctional type (JEB) JEB may be generalized or localized, with some uncommon orms associated with pyloric atresia or respiratory or renal involvement. Blisters in this orm o epidermolysis bullosa develop within the lamina lucida o the skin basement membrane zone. T e in ant in Fig. 17-20 has severe, generalized JEB, ormerly known as the Herlitz type. Blisters and erosions were present at birth. Patients with the severe, generalized orm o JEB develop scarring, have dystrophic or absent nails, and develop exuberant granulation tissue.

Fig ure 17-21

Fig ure 17-22

Epidermolysis bullosa, junctional type (JEB) Chronic, nonhealing erosions associated with granulation tissue tends to be most severe around the nose and mouth, and it is not unusual or granulation tissue to cause obstruction o the nares. Mucosal involvement o the both, the oral cavity and the gastrointestinal tract, lead to poor eeding, malnutrition, anemia, and growth retardation.

T ere is blister ormation surrounding a nonhealing erosion in Fig. 17-22. Scalp erosions and chronic paronychia with nail loss are common. T e prognosis varies according to disease severity, but in the severe JEB type there is a high rate o mortality during early childhood. In the generalized intermediate type, ormerly known as the non-Herlitz type, there is absent to rare development o granulation tissue.

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Fig ure 17-23

Fig ure 17-24

Epidermolysis bullosa recessive dystrophic type (RDEB) T e dystrophic orms o epidermolysis bullosa are characterized by blister ormation in the sublamina densa o the dermis, associated with de ects on collagen VII. T ere are recessive and dominant dystrophic orms. Epidermolysis bullosa recessive dystrophic type (RDEB) may be generalized o localized. T e generalized orm o RDEB is a multisystem disease characterized by chronic and recurrent blistering that leads to scarring as well as the development o chronic, nonhealing wounds.

T e disorder o en leads to severe disability, inter ering with normal growth and development. Blister ormation begins at or shortly a er birth, and intraoral involvement may lead to early eeding dif culties. Normal handling o the in ant with this disease results in the ormation o bullae that quickly evolve into ulcerations. T e typical blisters, crusts, and erosions o RDEB are seen in Figs. 17-23 and 17-24.

Fig ure 17-25

Fig ure 17-26

Epidermolysis bullosa recessive dystrophic type (RDEB) Figure 17-25 shows a oot with complete destruction o the toenails, induced syndactyly o the ourth and h toes, and typical wrinkled scarring o the skin sur ace. Appropriate care or the child with epidermolysis bullosa must begin in the nursery and involves the use o topical dressings to protect eroded skin and the care ul handling o the in ant to avoid new blister ormation. Subsequent management includes e orts to minimize skin trauma, good dental and ophthalmic care, and care ul attention to diet and nutrition.

Epidermolysis bullosa dominant dystrophic type, generalized (DDEB) Figure 17-26 shows a speci c orm o DDEB which was previously known as albopapuloid or Pasini type. T is orm o epidermolysis bullosa presents with generalized blistering at birth. During puberty, patients may develop distinctive ivory-white ollicular papules on the chest and lower back. T e prognosis tends to be good.

Se ction 1 7 . Bullous , Pus tular, and Ulce rating Dis e as e s

215

Fig ure 17-27

Fig ure 17-28

Dystrophic epidermolysis bullosa, dominant type (DDEB) Figure 17-27 illustrates another patient with a dominant orm o scarring epidermolysis bullosa. Previously known as the Cockayne- ouraine type, this orm tends to begin during in ancy and is associated with widespread blistering and milia ormation. Shown here are a number o tense bullae on the exor sur ace o the arm. Mouth involvement and nail dystrophy may also occur.

Some orms o DDEB have blistering limited to the elbows, hands, knees, and eet. T e blisters heal with scarring. Figure 17-28 reveals atrophic scarring, scaling, and hemorrhagic bullae on the knees and pretibial area.

Fig ure 17-29

Fig ure 17-30

Dystrophic epidermolysis bullosa, dominant type (DDEB) Figure 17-29 illustrates atrophic scarring on the knees. Patients with localized orms o DDEB also develop a nail dystrophy secondary to the blistering, at times leading to loss o the ngernails and toenails.

Epidermolysis bullosa with congenital absence of skin T ere are patients born with well-de ned areas o absence o skin limited to the legs and eet. In some cases, this occurrence is amilial. T is condition may represent a localized type o aplasia cutis congenita or o dominant dystrophic epidemolysis bullosa. Atrophic scarring is associated with this presentation.

216

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Fig ure 17-31

Fig ure 17-32

Dermatitis herpetiformis T is intensely pruritic papulovesicular eruption is airly unusual during childhood. Figure 17-31 shows the symmetrical distribution o the grouped, excoriated lesions on the extensor extremities and buttocks. Additional papules and vesicles on the upper chest and ace are seen in Fig. 17-32. Dermatitis herpeti ormis a ects both the skin and the gastrointestinal tract. T e majority o children with this disorder have a gluten-sensitive enteropathy.

T is may lead to diarrhea and malabsorption, or it may be evidenced only by villous atrophy on jejunal biopsy. T e approaches to treatment o this disease include sul apyridine or dapsone and a gluten- ree diet. As mentioned above, children treated with dapsone or sul apyridine must be care ully monitored or the development o hemolytic anemia. Adherence to a diet that contains no gluten is dif cult but, i accomplished, will o en lead to prolonged remission.

Fig ure 17-33

Dermatitis herpetiformis Figure 17-33 shows highly characteristic distribution o lesions o dermatitis herpeti ormis on the back. Because o the intense pruritus, excoriations tend to outnumber the primary papulovesicles. Direct immuno uorescence o perilesional skin is an e ective means o con rming the diagnosis o dermatitis herpeti ormis. ypically, there are granular deposits o IgA at the tips o the dermal papillae.

Se ction 1 7 . Bullous , Pus tular, and Ulce rating Dis e as e s

217

Fig ure 17-34

Fig ure 17-35

Linear Ig A dermatosis (chronic bullous dermatosis of childhood) T is is a distinct blistering disease that occurs exclusively during childhood, most commonly during the rst 5 years o li e. T e disorder is characterized by large, tense bullae that tend to occur in the genital area, lower abdomen and back, and lower extremities. T e degree o pruritus is variable. Figure 17-34 illustrates the particular ashion in which the blisters tend to cluster. Note the circular or oval con guration o blisters.

As an early lesion heals with crusting and hyperpigmentation, new lesions arise in a string-o -pearls or rosette-like pattern along the periphery. T e blisters themselves may be elongated or sausage-shaped. Figure 17-35 shows the clustering o tense blisters on the inner thighs. T is is probably the most common location or linear IgA dermatosis.

Fig ure 17-36

Fig ure 17-37

Linear IgA dermatosis (chronic bullous dermatosis of childhood) Figures 17-36 and 17-37 show the annular and polycyclic clustering o blisters that is so typical o this disorder. Biopsy o a lesion reveals a subepidermal blister with an in ltrate o neutrophils and/or eosinophils. Immuno uorescence is more distinctive and usually shows a linear deposit o IgA along the basement membrane (in the lamina lucida). In most patients, there are also circulating IgA antibodies directed against the basement membrane.

For most a ected children this is a sel -limited disease and it resolves spontaneously over a period o several years. Sul apyridine and dapsone are the most e ective therapies. T ese drugs have hemolytic activity, and the patients must be monitored or induced anemia and methemoglobinemia. A test or glucose-6phosphate dehydrogenase should be done be ore initiation o therapy, and hemoglobin determinations should be made subsequently until stabilization occurs.

SECTION

18 Cutaneous Mani estations o HIV Disease

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Fig ure 18-1

Fig ure 18-2

Cutaneous mani estations o HIV in ection (chronic varicella zoster in ection) Figure 18-1 illustrates a orm o in ection with varicella zoster virus that is unique to patients with immune suppression rom HIV. T e 6-year-old child pictured here developed recurrent vesicular and ulcerative lesions o the trunk and extremities ollowing an episode o chickenpox. T e lesions contain numerous multinucleated giant cells and are culture-positive or varicella zoster virus.

Cutaneous mani estations o HIV in ection (herpes zoster in ection) In addition to chronic in ections with varicella zoster, many patients develop unusual orms o herpes zoster in ection. T ese patients also develop prolonged episodes o shingles that do not respond quickly to appropriate antiviral agents; they may also develop generalized varicella zoster in ections.

Fig ure 18-3

Fig ure 18-4

Cutaneous mani estations o HIV in ection (scarring rom herpes zoster) Figure 18-3 shows a 3-year-old girl developed herpes zoster as an early mani estation o her immune de ciency. Despite therapy with intravenous acyclovir, severe scarring resulted. Herpes zoster generally occurs more requently in children who have had chickenpox very early in li e. Although herpes zoster is certainly seen in the healthy child, its occurrence in a child who is at risk or HIV in ection should signal concern.

Cutaneous mani estations o HIV in ection (candidal paronychias and nail dystrophy) Candidiasis is the most common mucocutaneous mani estation o pediatric HIV in ection. Children with AIDS or lesser orms o HIV-related disease requently develop oral thrush, which recurs or persists despite topical anti ungal therapy. Recalcitrant in ections o the diaper area and neck olds are also common. Illustrated in Fig. 18-4 are chronic paronychias with a resultant nail dystrophy.

Se ction 1 8 . Cutane ous Manife s tations of HIV Dis e as e

221

Fig ure 18-5

Fig ure 18-6

Cutaneous mani estations o HIV in ection (drug eruption) Drug eruptions, usually due to therapy with trimethoprimsul amethoxazole, are particularly common among children with HIV in ection. T is child shown in Fig. 18-5 developed Steven-Johnson syndrome, which is a airly requent occurrence among children in ected with HIV. T e requency o drug eruptions in children with AIDS illustrates the complex e ect o HIV on the immune system.

Cutaneous mani estations o HIV in ection (molluscum contagiosum) Children in general are prone to in ection with the virus causing molluscum contagiosum. Children in ected with the HIV are more likely to develop persistent, widespread eruptions due to molluscum contagiosum, and are more likely to develop the numerous giant lesions that are pictured in Fig. 18-6.

Fig ure 18-7

Fig ure 18-8

Cutaneous mani estations o HIV in ection (chronic herpetic gingivostomatitis) Children with AIDS requently su er rom persistent herpetic in ections o the mucous membranes or skin. T e gingivostomatitis shown in Fig. 18-7 would be typical o primary in ection in a healthy child. One would then expect recurrences to be considerably milder. By contrast, the child with HIV in ection may have recurrent episodes o gingivostomatitis or may develop in ection that does not respond at all to acyclovir or related drugs.

Cutaneous mani estations o HIV in ection (seborrheic dermatitis) Seborrheic dermatitis occurs with particular severity in individuals who are in ected with HIV. T e in antile orm consists o severe scaling and erythema o the scalp and exural areas and sometimes o the entire skin sur ace. Illustrated in Fig. 18-8 is an older child with recalcitrant in ammation behind the ears. She also had crusting and scaling o the entire scalp.

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Fig ure 18-9

Fig ure 18-10

Cutaneous mani estations o HIV in ection (condylomata acuminata) Children who are in ected with the HIV are prone to a wide variety o cutaneous viral in ections. Lesions caused by human papillomavirus (HPV) may be unusually widespread and persistent. A case o condylomata acuminata in a 1-year-old girl is illustrated in Fig. 18-9.

Cutaneous mani estations o HIV in ection (widespread f at warts) T ese coalescent shiny gray macules and slightly elevated plaques are typical o a distinctive clinical mani estation o HPV in ection in children with HIV/AIDS. T ere is a strong tendency or the lesions to Koebnerize (develop in a linear array) where the skin has been scratched. Unlike other cutaneous mani estations o HIV in ection, these warts tend not to resolve during success ul antiretroviral therapy.

Fig ure 18-11

Fig ure 18-12

Cutaneous mani estations o HIV in ection (psoriasis) Psoriasis is signi cantly more common among children and adolescents with HIV in ection, and of en occurs in the absence o a amily history. T e two patients shown in Figs. 18-11 and 18-12 have severe and generalized plaque psoriasis.

Eryrthrodermic and inverse psoriasis are also seen in the context o HIV in ection, and some patients simultaneously develop several morphologic types. Most patients with HIV-related psoriasis improve with success ul antiretroviral therapy.

SECTION

19 Cutaneous Mani estations o Systemic Disease

224

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Fig ure 19-1

Fig ure 19-2

Systemic lupus erythematosus Figures 19-1 and 19-2 illustrate cutaneous involvement o systemic lupus erythematosus (SLE) in the classic butter y pattern on the ace. T is macular and intensely erythematous eruption is requently aggravated by sun exposure and may are with other symptoms o systemic disease.

T is autoimmune disease o unknown etiology a ects almost every organ system. T e most common ndings in the child with SLE are ever, arthralgias, and arthritis. In addition, pleuritis, pericarditis, and central nervous system involvement are requently seen in children with SLE. Lupus nephritis develops in the vast majority o a ected children and may eventually cause renal ailure.

Fig ure 19-3

Fig ure 19-4

Systemic lupus erythematosus T ese gures illustrate additional cutaneous eatures o SLE. he lesions on the back (Fig. 19-3) and the hands (Fig. 19-4) are intensely erythematous macules and slightly edematous papules and plaques. Childhood SLE occurs most requently during adolescence and is more common among A rican-Americans and among girls.

Clinical criteria include rash (either malar or discoid), photosensitivity, oral ulcers, and disease o the joints, lungs, kidneys, and central nervous system.

Se ction 1 9 . Cutane ous Manife s tations of Sys te m ic Dis e as e

225

Fig ure 19-5

Fig ure 19-6

Systemic lupus erythematosus Figure 19-5 shows the temporary alopecia that is also a hallmark o SLE. T e single most reliable laboratory test or SLE is antinuclear antibody which will be positive in almost every case o childhood SLE. Other laboratory criteria include leukopenia, thrombocytopenia, and the presence o antinative DNA antibodies.

Involvement o the distal ngers is a common occurrence in children with SLE. Findings include telangiectasia o the nail olds, splinter hemorrhages, and digital in arcts as shown in Fig. 19-6.

Fig ure 19-7

Systemic lupus erythematosus vasculitis Cutaneous vasculitis occurs commonly in patients with SLE. T e vasculitis may lead to ulceration as shown in Fig. 19-7.

226

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Fig ure 19-8

Fig ure 19-9

Neonatal lupus erythematosus T is condition is primarily due to the transplacental passage mainly o anti-Ro (anti-SS-A) antibodies rom mother to in ant. Maternal anti-La (anti-SS-B) and anti-RNP antibodies may be implicated. T e mother may hersel su er rom a orm o connective tissue disease or may be completely asymptomatic.

T e in ants pictured in Figs. 19-8 and 19-9 were born with these atrophic and telangiectatic plaques on the ace. Symmetrical involvement o the skin around the eyes is one common pattern. T e skin lesions, which are slightly more common in girls, tend to resolve without scarring. Non uorinated topical steroids and avoidance o the sun are the only treatments that may be required.

Fig ure 19-10

Fig ure 19-11

Neonatal lupus erythematosus Figure 19-11 shows the atrophic telangiectatic changes that are most o en seen on the temple and scalp, and which may lead to permanent alopecia.

A requent complication o neonatal lupus is congenital heart block, which may be ound in utero. T is situation is potentially li e threatening and may sometimes require implantation o a pacemaker. Although neonatal lupus is sel -limited, there are reports o a ected in ants going on to develop connective tissue disease during adolescence.

Se ction 1 9 . Cutane ous Manife s tations of Sys te m ic Dis e as e

227

Fig ure 19-12

Fig ure 19-13

Discoid lupus erythematosus Figures 19-12 and 19-13 show the characteristic plaques o discoid lupus erythematosus. T ere is a violaceous hue at the border and central atrophy with scarring. Similar involvement in the scalp can cause permanent alopecia.

T e majority o children with discoid lupus erythematosus do not go on to develop systemic disease. However, discoid lesions may be a cutaneous mani estation o SLE and children with this disorder should be monitored or the development o systemic disease.

Fig ure 19-14

Fig ure 19-15

Discoid lupus erythematosus reatment consists o avoidance o the sun or the use o sunscreens, antimalarial agents, and topical or intralesional corticosteroids.

Illustrated in Fig. 19-15 is a plaque o discoid lupus erythematosus involving the pinna o the ear. Lesions are commonly ound in this location, and also within the conchal bowl o the ear.

228

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Fig ure 19-16

Fig ure 19-17

Dermatomyositis T is collagen vascular disease is characterized by a combination o symmetrical proximal upper and lower extremity muscle weakness and cutaneous disease. Early symptoms o muscle involvement include general malaise, dif culty in playing or climbing stairs, myalgias, and muscle tenderness.

Figures 19-16 and 19-17 show symmetrical, intense erythema and edema o the ace. T e violaceous color o the periorbital rash is sometimes described as heliotrope in hue; the edema is so intense that it makes the ace look pu y.

Fig ure 19-18

Fig ure 19-19

Dermatomyositis Erythematous and edematous papules and plaques may also be present on the elbows and knees. Scaling may also be present, as illustrated in Fig. 19-18, and this mani estation o dermatomyositis may be con used with psoriasis. Psoriasi orm scalp involvement may also be a presenting eature.

Figure 19-19 illustrates typical papules and plaques located on the dorsum o the hand.

Se ction 1 9 . Cutane ous Manife s tations of Sys te m ic Dis e as e

229

Fig ure 19-20

Fig ure 19-21

Dermatomyositis Figures 19-20 through 19-22 illustrate involvement o the dorsal hands. Gottron’s papules are classically situated over the knuckle joints, and unlike other dermatoses involving the hands, conspicuously spare the skin between those joints.

In skin o color, the papules are signi cantly less erythematous, but show the same characteristic morphology and distribution.

Fig ure 19-22

Fig ure 19-23

Dermatomyositis T e diagnosis o dermatomyositis is usually made on the basis o elevated muscle enzymes (especially creatinine phosphokinase and aldolase), magnetic resonance imaging (MRI), and muscle biopsy i necessary. Skin biopsy tends not to be help ul in di erentiating dermatomyositis rom other collagen vascular disease. Some patients may develop typical Gottron’s papules in the absence o muscle disease (dermatomyositis sine myositis).

Figure 19-23 shows erythema and telangiectasia in the proximal nail old. Early and aggressive therapy is vital in the treatment o the muscle disease and in the prevention o calcinosis. In contrast to the adult orm o the disease, childhood dermatomyositis is not associated with malignancy.

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Fig ure 19-24

Fig ure 19-25

Dermatomyositis Although Gottron’s papules are most commonly seen on the dorsal hands, they also may occur on the bilateral elbows. In that case, they should be distinguished rom erythema elevatum diutinum ( igures 16-27 and 16-28) rictional lichenoid dermatitis ( gures 9-20 and 9-21) and an id reaction to nickel.

An additional cutaneous nding, which is particularly characteristic o childhood dermatomyositis, is calcinosis cutis. T is may present as rm, super cial nodules or plaques or as pain ul deposits o calcium in muscle or ascia. reatment or this troubling disorder is challenging.

Fig ure 19-26

Fig ure 19-27

Scleroderma (progressive systemic sclerosis) T is is a multisystem connective tissue disease that is relatively rare in childhood. Patients may su er rom arthritis and muscle weakness and rom the results o pulmonary, esophageal, cardiac, and renal involvement. T e induration o the skin on the ace and hands is shown in Fig. 19-26. T e pallor o the knuckles gives a sense o the in exibility o the ngers.

Figure 19-27 shows the pallor o Raynaud phenomenon and telangiectasia. T e patient also has scleroderma o the hands, orearms, and ace. I calcinosis cutis were also present, this type o case would be designated as the CRES syndrome, characterized by calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Such a combination is the most severe o the cutaneous possibilities o scleroderma.

Se ction 1 9 . Cutane ous Manife s tations of Sys te m ic Dis e as e

231

Fig ure 19-28

Fig ure 19-29

Cutaneous expression of rheumatic fever Erythema marginatum is a speci c cutaneous expression o rheumatic ever. T e lesions, transient and recurrent over days and weeks, may precede cardiac or joint symptoms or signs.

Cutaneous expression of rheumatoid arthritis Patients with rheumatoid arthritis may develop dermographism or edematous erythema provoked by scratching. T ese lesions are most evident during a ebrile period o the illness. Figure 19-29 shows persistent and transient stripes o erythema and edema on the back and buttocks.

Fig ure 19-30

Fig ure 19-31

Cutaneous expressions of polyarteritis nodosa T is multisystem disease is a orm o necrotizing vasculitis that a ects small and medium-sized arteries. Frequent areas o systemic involvement are the gastrointestinal tract and kidneys. Palpable purpura is the most common cutaneous mani estation o this disease. In addition, there may be necrotic ulcers and distal gangrene.

ender cutaneous nodules, which may be transient, are occasionally seen (Fig. 19-30), but these are more characteristic o benign cutaneous periarteritis nodosa, which has no systemic mani estations. A livedo reticularis pattern is o en seen in this condition (Fig. 19-31).

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Fig ure 19-32

Fig ure 19-33

Sarcoidosis T is is a disease o unknown etiology that is characterized by the ormation o noncaseating granulomas in many di erent organ systems. It is unusual in children but it does occur. During childhood, the most common presenting signs and symptoms are lymphadenopathy, uveitis, and cutaneous disease. In very young children, arthritis, eye disease, and skin disease tend to predominate. Figure 19-32 illustrates the erythematous and esh-colored papules that may be the sole cutaneous mani estation o sarcoidosis.

Sarcoidosis is more common among A rican-Americans. Children and adolescents with sarcoidosis may also have weight loss, ever, and respiratory involvement. Disorders o calcium metabolism are evidenced by hypercalcemia and hypercalciuria. Systemic steroids remain the treatment o choice in this disease. Illustrated here are highly characteristic papules o sarcoidosis on and around the eyelids. Similar lesions are common around the nose and mouth. Investigation or systemic disease should include X-rays o the lungs and pulmonary unction tests. T e eyes should be examined or uveitis and lacrimal gland enlargement.

Fig ure 19-34

Fig ure 19-35

Pernio Children with pernio develop recurrent pain ul or pruritic, erythematous or violaceous nodules or plaques on the distal ngers or toes. T e development o lesions is usually related to cold exposure, and episodes last 2 to 3 weeks.

In the majority o patients, pernio is an isolated benign nding However, less commonly, it is a symptom o cryoglobulinemia, antiphospholipid antibody syndrome, connective tissue disease, or hematologic malignancy. T e best treatment is prevention: keeping ngers and toes warm in cold weather.

Se ction 1 9 . Cutane ous Manife s tations of Sys te m ic Dis e as e

233

Fig ure 19-36

Fig ure 19-37

Cutaneous manifestations of Crohn disease T is chronic in ammatory bowel disease has several cutaneous mani estations. Most common are perianal ssures, stulas, and granulomatous nodules. T e exophytic lesions shown in Fig. 19-36 could easily be con used with condylomata acuminata. Metastatic Crohn disease may present with ulcerations in intertriginous olds. Children with Crohn disease may also su er rom erythema nodosum and aphthous ulcers.

Crohn disease may also present with erythema and edema o the labia majora, which can progresses to extensive ulcer ormation. T is orm o skin involvement, which may be unilateral or bilateral, may develop be ore or a er the gastrointestinal symptoms.

Fig ure 19-38

Fig ure 19-39

Pyoderma gangrenosum T is orm o gradually enlarging necrotic ulceration usually involves the distal lower extremity. ypical characteristics include a purulent base and a purplish undermined border. Etiologies include in ammatory bowel disease, chronic active hepatitis, and Behçet syndrome. Arthritis may be an associated nding.

reatment consists o bed rest, local care, and may include injection o intralesional steroids along the border o the lesion. Oral corticosteroids, cyclosporin A, dapsone, mycophenolate mo etil, and azathioprine are additional therapies, and the anti- NF agents may also be use ul.

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Fig ure 19-40

Fig ure 19-41

Kawasaki disease T is childhood disease o unknown etiology is of cially diagnosed by the presence o high ever lasting or 5 or more days, and our o the ollowing criteria: conjunctival injection (Fig. 19-40), mucous membrane changes (Figs. 19-41 and 19-42), erythema or edema o the palms and soles, rash (Fig. 19-43), and cervical lymphadenopathy.

T e clinical eatures o Kawasaki disease are o en evanescent, and, requently the diagnosis is only considered during the second week o illness. For this reason, care ul attention to the recent history o oral and cutaneous ndings is extremely important.

Fig ure 19-42

Fig ure 19-43

Kawasaki disease Pictured in Fig. 19-42 is a child with strawberry tongue, a nding also seen in scarlet ever and toxic shock syndrome. Systemic aspects o Kawasaki disease include arthritis, aseptic meningitis, and hydrops o the gallbladder.

Coronary artery aneurysms develop in 20 percent o patients who are not treated, and some children go on to develop myocardial in arction. Current therapy, consisting o aspirin and intravenous gamma globulin, reduces the incidence o cardiac disease.

Se ction 1 9 . Cutane ous Manife s tations of Sys te m ic Dis e as e

235

Fig ure 19-44

Fig ure 19-45

Kawasaki disease A common cutaneous nding during the second week o the illness is desquamation o the palms and soles beginning around the distal portion o the digits and then extending upward.

Figure 19-45 illustrates the desquamation o the diaper area that requently occurs in Kawasaki disease. All children with a history o Kawasaki disease should be ollowed with serial two-dimensional echocardiography to monitor or the progression o cardiac involvement.

Fig ure 19-46

Fig ure 19-47

Necrobiosis lipoidica T is condition usually occurs in patients with diabetes but is also rarely associated with rheumatoid arthritis. T e most common location is on the shins, and the condition is o en bilateral. T e lesions are usually asymptomatic, but may also be tender. T e lesion may be erythematous at rst, but eventually evolves into an indurated plaque with a yellowish or brown tint.

T is condition has some histologic and clinical resemblance to granuloma annulare (Figs. 20-24 to 20-29), but the two conditions are distinct. T e lesions o necrobiosis lipoidica are generally much larger than those o granuloma annulare and patients with necrobiosis lipoidica can develop atrophy and scarring, as illustrated in Fig. 19-47.

SECTION

20 Disorders o the Dermis (In ltrates, Atrophies, and Nodules)

238

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Fig ure 20-1

Fig ure 20-2

Morphea T is condition is characterized by discrete areas o hardened and discolored skin. T e areas vary in size and shape, and may be single or multiple. Morphea is also sometimes re erred to as localized scleroderma, but this condition must not be con used with scleroderma, a generalized disease with organ involvement. Families must be cautioned o this important di erence so that their internet searches do not lead to unnecessary worry.

T e solitary or individual lesion is a circle or oval o rm skin that is whitish, slightly depressed, and sometimes surrounded by a di erent color—lilac or purple. A lesion with a prominent purple vascular border is illustrated in Fig. 20-2. T ere may be only one such lesion, or multiple smaller and larger lesions covering large parts o the skin sur ace. T e course o the condition is variable. Spontaneous recovery in children is common, but a wide variety o topical and systemic therapies have been recommended or lesions that cause signi cant problems with respect to appearance and unction.

Fig ure 20-3

Fig ure 20-4

Morphea (linear) Figure 20-3 shows a type o morphea that is linear in shape and situated on the lateral sur ace o the leg. Again the a ected skin is hard, slightly depressed, and dyschromic. Extensive areas o linear morphea are the most di cult to treat. T ere is a tendency toward permanent de ormity. In this case, linear morphea appears to ollow the lines o Blaschko, and suggests the possibility o mosaicism.

When the lesions cross over a joint, the rapid development o atrophy or contractures may occur. In those cases, systemic therapy, usually with pulsed intravenous steroids and methotrexate, is required.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

239

Fig ure 20-5

Fig ure 20-6

Morphea (linear) T is is an example o linear morphea involving the lip, chin, and neck. T e skin is hard to the touch, and shiny with prominent vasculature. Rarely, linear morphea occurring in this location may lead to involvement o the gums and tongue.

Morphea (linear, en coup de sabre) T is term re ers to linear morphea occurring on the orehead and scalp. Rarely, the central nervous system is a ected. Neurologic mani estations include seizures, ocal neurologic de cits, and movement disorders. MRI in a ected children shows some areas o 2 hyperintensity and evidence o ocal tissue atrophy in the brain.

Fig ure 20-7

Fig ure 20-8

Morphea Illustrated in Fig. 20-7 is a lesion o morphea that is resolving. T e rst sign o improvement is so ening in the area o involvement. In this patient, the lesion is becoming so er, and turning brown. In the center there is still an area o sclerosis.

Figure 20-8 shows a lesion that is reddish-brown and is now level with the surrounding skin. It will eventually completely resolve or leave minor dyschromia. T e cause o morphea is unknown.

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Fig ure 20-9

Fig ure 20-10

Generalized morphea Sometimes morphea can be more extensive and involve large areas o the skin sur ace. T e lesions begin as erythematous to violaceous plaques and become sclerotic with a central ivory color. Sometimes only hyperpigmentation can be discerned.

T ese lesions may enlarge peripherally, becoming quite extensive. When involvement occurs on the extremities, contractures and atrophy may result. Scarring may result in disability, but these patients do not have the internal organ involvement that is seen in scleroderma (systemic sclerosis).

Fig ure 20-11

Atrophoderma (Pasini-Pierini) T is condition is most common in adolescent girls, and its etiology is completely unknown. Characteristically, there develop single or multiple well-circumscribed, slightly hyperpigmented areas o cutaneous atrophy, with a sharp “cli -drop” border. T e lesions tend to enlarge slowly and then persist. Some believe that this orm o atrophoderma represents a variant o morphea, in which atrophy is more evident than is sclerosis.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

241

Fig ure 20-12

Fig ure 20-13

Lichen sclerosus et atrophicus Figures 20-12 and 20-13 illustrate lichen sclerosus et atrophicus in its most typical location. T is cutaneous disorder is more common in girls and is usually con ned to the skin surrounding the anogenital region. T e onset o the disease may be accompanied by pruritus, burning, constipation, or vaginal discharge, or it may be completely asymptomatic. Porcelain-colored or slightly erythematous macules gradually coalesce to orm plaques.

T e end result is an area o shiny atrophy in an hourglass shape around the rectum and vagina. Childhood lichen sclerosus et atrophicus tends to be a sel -limited disease, with improvement occurring at the time o puberty. It may persist into adulthood. T e use o topical corticosteroids provides symptomatic relie and may hasten the resolution o the process.

Fig ure 20-14

Fig ure 20-15

Lichen sclerosus et atrophicus Figure 20-14 shows the discrete and conf uent ivory-colored macules that are typical o extragenital lichen sclerosus et atrophicus. Involvement as shown here, on the skin behind the ear is unusual. Extragenital lesions tend to be asymptomatic.

Figure 20-15 illustrates lichen sclerosus et atrophicus involving the ankle, which is not an unusual location. T e classic lesion contains numerous white round and oval-shaped macules that, in some cases, orm areas o conf uence. Rarely, extragenital lichen sclerosus is widespread and dis guring.

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Fig ure 20-16

Fig ure 20-17

Lichen sclerosus et atrophicus (Balanitis xerotica obliterans) T is is a orm o lichen sclerosus et atrophicus a ecting the penis. It usually presents as recurrent balanitis, associated with redness, tightening, and ssuring o the oreskin. Eventually, the disease process may result in phimosis and di culty in urination. In many cases, circumcision is either very help ul or curative. However, involvement o the glans penis may persist a er surgery. Although this disorder is generally rare, it is not an unusual nding among young boys who present with phimosis.

Anetoderma (macular atrophy) T e term anetoderma, meaning slack skin, is used to describe a orm o cutaneous atrophy. T e classic lesion is a macule o atrophic and wrinkled skin. T e lesions o anteoderma may be preceded by inf ammation, or may occur de novo on normal skin. Anetoderma may arise without a known cause, or it may be a sequelae o syphilis, or lupus erythematosus.

Fig ure 20-18

Fig ure 20-18

A Anetoderma Illustrated in Fig. 20-18 is a lesion o anetoderma on the arm o a teenage girl. Direct pressure with a wooden

B applicator causes an inpouching o the involved skin.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

243

Fig ure 20-19

Fig ure 20-20

Striae distensae Stretch marks are parallel streaks o glossy and erythematous skin, that gradually become hypopigmented and scar-like in appearance. T e stretch marks o en have a di erent texture rom normal skin and may be slightly depressed. Striae are commonly seen in healthy adolescents and may be ound on the back, around the breasts, upper arms, hips, inner thighs, and around the popliteal ossae.

Other causes o striae include pregnancy and rapid weight gain. Striae may also be seen in children with Cushing syndrome. Athletes, particularly weight li ers, sometimes develop striae distensae. In Fig. 20-19, one sees striae on the back that may well be o a weight li er. Figure 20-20 shows striae and telangiectasia as a side e ect o topical corticosteroids, which is most requent in older children and adolescents.

Fig ure 20-21

Striae distensae T is is an example o striae resulting rom the use o topical corticosteroids in the antecubetal ossa, a typical area o involvement o atopic dermatitis. Older children and teenagers must be made particularly aware o this potential side e ect, and should be cautioned against using stronger topical steroids on the inner thighs and axillae.

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Fig ure 20-22

Fig ure 20-23

Connective tissue nevus T is group o hamartomas may be composed o various elements o the extracellular connective tissue: collagen, elastic bers, and glycosaminoglycans. One example o the collagen variety is the shagreen patch, a cutaneous mani estation o tuberous sclerosis (Fig 14-69). T ese yellow or orange plaques o coalescent papules are o en seen in the lumbosacral area. Other varieties o connective tissue nevus are not associated with tuberous sclerosis and may be seen elsewhere on the body.

Particularly, collagenomas may be seen in solitary (Figs. 20-22 and 20-23), eruptive, and multiple orms. T e last o these is a amilial disorder. Elastic bers are ound in isolated elastomas and in the small yellowish papules o the autosomal dominant Buschke-Ollendor syndrome (Fig 14-71). T e latter is also characterized by asymptomatic sclerotic densities o the bones.

Fig ure 20-24

Fig ure 20-25

Granuloma annulare T is benign condition is characterized by one or more circular plaques consisting o rings o papules around a depressed center. T e hands and eet are the most common location or granuloma annulare. T e majority o patients have only one or several lesions, but occasionally, eruptive and widespread lesions occur.

Granuloma annulare, when it occurs in children, is not associated with any other disease, nor is there any known cause. T e condition is symptomless. Lesions may persist or months or years be ore response to therapy or spontaneous resolution.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

245

Fig ure 20-26

Fig ure 20-27

Granuloma annulare Figure 20-26 illustrates a very typical lesion o granuloma annulare in a common location. Because o the circular appearance with central clearing, granuloma annulare is o en mistaken or a ungal in ection. However, the indurated nature o the periphery and the absence o scale make the diagnosis obvious in most cases.

Subcutaneous granuloma annulare T is orm o granuloma annulare presents with deep nodules. In some cases, as illustrated in Fig. 20-27, a more typical annular lesion may surround the nodule. T ese lesions are most requently localized to the digits, scalp, dorsa o the eet, or anterior tibial region.

Fig ure 20-28

Fig ure 20-29

Subcutaneous granuloma annulare T is patient in Fig. 20-28 developed a subcutaneous nodule on the dorsum o the oot. T e lesion is not pain ul or tender, and has a rubbery consistency. In some cases, the diagnosis o subcutaneous granluloma annulare can be con rmed by the presence o typical annular lesions in other locations.

Eruptive granuloma annulare T is generalized orm o the disease is more unusual. It presents with numerous f esh-colored or pink-red papules. In some cases, they coalesce into round or reticulate clusters. A subtle annularity, especially noted on palpation o a lesion, may be the clue to diagnosis. In most children, eruptive granuloma annulare avors the trunk, but it is occasionally seen in a photodistribution. In children, this disorder does not appear to be associated with any systemic illness.

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Fig ure 20-30

Fig ure 20-31

Juvenile xanthogranuloma T is is a common and completely benign cutaneous nodule. ypically, a juvenile xanthogranuloma is rm and dome-shaped. At rst, the lesion is reddish as in Fig. 20-30, but develops a airly typical orange-brown hue over time (Figs. 20-31 to 20-33).

T e bright orange color seen in this lesion is virtually pathognomonic or juvenile xanthogranuloma. Most juvenile xanthogranulomas are located on the head or neck, but the lesions sometimes occur on the trunk or extremities. T ey may be present at birth, but most develop during the rst year o li e.

Fig ure 20-32

Fig ure 20-33

Juvenile xanthogranuloma Juvenile xanthogranuloma is not associated with abnormalities in serum cholesterol or triglycerides, and the individual lesions undergo spontaneous involution, usually over a period o 1 to 2 years.

A diagnostic biopsy analysis is sometimes needed; surgical intervention is possible, but certainly not required. Larger lesions may resolve with an area o atrophic skin or a hyperpigmented or hypopigmented macule.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

247

Fig ure 20-34

Fig ure 20-35

Juvenile xanthogranuloma Pictured in Fig. 20-34 is a solitary nodular acial lesion. Multiple juvenile xanthogranulomas on the skin may be accompanied by intraocular lesions. For this reason, the physician must pay care ul attention to the examination o the eyes and consider an ophthalmologic re erral or the child with multiple lesions. Certainly, an abnormality in the color o the iris or an enlargement o the globe should trigger a prompt re erral.

Rarely, as illustrated in Fig. 20-35, a juvenile xanthogranuloma can grow very large. In this case, surgical excision may well be desirable approach.

Fig ure 20-36

Fig ure 20-37

Juvenile xanthogranuloma Figure 20-36 shows our lesions. It is somewhat more common to see multiple juvenile xanthogranulomas than a solitary lesion. Rarely, they may number in the hundreds. As mentioned, juvenile xanthogranuloma o the eye is the most common complication o this generally benign and sel -limited condition.

When multiple small juvenile xanthogranulomas are seen in children with neuro bromatosis, these patients have an additional tendency toward the development o juvenile chronic myelocytic leukemia. Rarely, as illustrated in Fig. 20-37, juvenile xanthogranulomas may become agminated and appear as a plaque.

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Fig ure 20-38

Fig ure 20-39

Dermato broma T ese are benign dermal nodules that represent a ocal proli eration o broblasts; the overlying epidermis is slightly thickened. T eir occurrence is not unusual in children and adolescents. Dermato bromas are rm and may be black, red, brown, or f esh-colored. T eir diameter generally ranges rom 0.5 to l.5 cm, although they may occasionally be larger. Dermato bromas may be solitary or multiple, and they develop either spontaneously or a er minor trauma to the skin, such as an insect bite.

Most are asymptomatic but sometimes dermato bromas may be pain ul on palpation. A very use ul diagnostic maneuver is executed by exerting lateral pressure on the lesion. T e skin overlying a dermato broma will requently dimple. Dermato bromas require surgical treatment only when they are o cosmetic concern to the patient. However, biopsy is occasionally required in order to con rm the diagnosis and to di erentiate it rom more serious disorders.

Fig ure 20-40

Fig ure 20-41

Granular cell tumor T ese are unusual nodular lesions that vary rom 0.5 to 3.0 cm in diameter. T ey may be single (Fig. 20-40) or multiple (Fig. 20-41) and are rm, elevated, and circumscribed. T e tongue is a common site. T e lesions pictured in both gures are airly typical o the cutaneous variety.

T e original name granular cell myoblastoma derived rom an early theory that these tumors arise rom immature striated muscle cells. In act, granular cell tumors are o neural derivation.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

249

Fig ure 20-42

Fig ure 20-43

Benign cephalic histiocytosis T is very distinctive eruption consists o numerous brownish-yellow macules and papules involving the head and neck. T e mucous membranes are spared. Benign cephalic histiocytosis usually begins during the rst 3 years o li e, and the lesions continue to evolve or several years. T e condition then resolves spontaneously during childhood but may leave small atrophic or pigmented scars.

Children with benign cephalic histiocytosis do not develop systemic disease and are not at risk or Langerhans cell disease. T is disorder can be diagnosed by skin biopsy with appropriate immunostains.

Fig ure 20-44

Fig ure 20-45

Keloids T e lesions pictured in Fig. 20-44 are typical o the dis guring changes that sometimes occur a er trauma to the skin. In children, a laceration, a surgical procedure, or a bout o chicken pox may be the inciting event or keloid ormation. T is gure shows a keloid that was caused by ear piercing. Keloid ormation is more common in A rican-American children, is very rare in in ants, and becomes somewhat more requent with increasing age. Keloids begin as rm, telangiectatic plaques conned to the site o the initial wound. Over time, they become less erythematous and extend beyond the site o injury.

Pruritus, burning, and hyperesthesia are requent complaints. In contrast to hypertrophic scars, keloids extend beyond the limits o the original scar and do not resolve spontaneously. In act, they may continue to grow over a period o years. T e intralesional injection o corticosteroids is currently the pre erred treatment or most keloids. Surgical excision will almost always result in recurrence but is somewhat more success ul when accompanied by the injection o corticosteroids during the process o healing.

250

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Fig ure 20-46

Fig ure 20-47

Keloids T e keloid pictured in Fig. 20-46 is a common variety that develops a er a thermal burn. T e irregular contour and crab-like projections are characteristic and illustrate the root o the word keloid: rom the Greek word chele, meaning crab’s claw. T e size and thickness o lesions o this type make treatment di cult. T e chest and back are also common sites or keloids o another cause, namely, inf ammatory acne. In that situation, repeated injections o intralesional corticosteroids are o en o cosmetic bene t.

Fibrous hamartoma o in ancy T is benign growth may be present at birth, or develop during the rst 2 years o li e. T e solitary subcutaneous nodule ranges rom 2 to 5 cm in diameter and is sometimes lumpy on palpation. In most cases, the lesion occurs on the axilla, shoulder, upper arm, and chest wall. Other possible sites are the inguinal region and the buttocks. Diagnosis depends on histopathological examination, and the treatment o choice is excision.

Fig ure 20-48

Fig ure 20-49

Digital brous tumor o childhood T is is a rare bromatosis that starts in in ants either as a single nodule or, less commonly, as several nodules. Parents may be alarmed by the rapid growth o the lesions. Digital brous tumors usually occur on the dorsal and sometimes on the lateral aspects o the distal phalanges o ngers and toes.

T ey may be globular, red, and smooth as illustrated in Fig. 20-48, or reddish-brown and convoluted, especially when they wrap around digits, as illustrated in Fig. 20-49. De ormity o the adjacent nail plate may occur. T e lesions are benign and have never been known to become malignant or metastasize.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

251

Fig ure 20-50

Fig ure 20-51

Digital brous tumor o childhood Recurrence is a common event a er surgical excision, but many such tumors will involute without treatment over a period o several years. In most cases, the best treatment is simple observation

Figures 20-50 and 20-51 are o the same patient, and illustrate the process o spontaneous involution over several years.

Fig ure 20-52

Fig ure 20-53

In antile myo bromatosis T e majority o lesions present at birth or shortly therea er. T ey may be super cial or may involve the subcutaneous tissue and muscle. T e typical lesion o in antile myo bromatosis is a rubbery or rm nodule (Fig. 20-52), sometimes measuring up to 7 cm in diameter (Fig. 20-53). Solitary lesions tend to occur on the head and neck, and resolve spontaneously, sometimes leaving areas o atrophy or pigmentary alteration.

Children with multiple lesions (the generalized orm) are particularly at risk or visceral and bone involvement. While the skin and bone lesions in these children also tend to disappear spontaneously, the visceral lesions (lungs, heart, and gastrointestinal tract) are a signi cant cause o morbidity and mortality.

252

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Fig ure 20-54

Fig ure 20-55

Mastocytoma T is is one o a group o disorders that are characterized by the accumulation o mast cells in the skin and sometimes in other organs. T e plaque shown in Fig. 20-54 represents a solitary mastocytoma, which occurs almost exclusively during in ancy. T is is most o en a reddish-brown or orange nodule or plaque that is rubbery in consistency. T ere is occasionally a history o repeated swelling or even blister ormation on the sur ace.

Pictured in Fig. 20-55 is the same patient as in the previous photo. When a solitary mastocytoma or a lesion o mastocytosis is rmly rubbed, the area will turn red, swell, and occasionally blister. T is diagnostic test is termed Darier’s sign and it is virtually diagnostic o mast cell disease. Mechanical trauma is thought to degranulate mast cells and cause a local release o histamine. Dermographism in uninvolved skin is not uncommon.

Fig ure 20-56

Fig ure 20-57

Mastocytosis Figure 20-56 shows the numerous macules and papules o reddish or brownish hue that are typical o urticaria pigmentosa. T is generalized orm is the most common type o mastocytosis, and it also usually develops during early childhood.

Figure 20-57 illustrates the typical lesions o generalized mastocytosis (urticaria pigmentosa). Numerous brown and orange papules are coalescing to orm plaques.

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

253

Fig ure 20-58

Fig ure 20-59

Mastocytosis (cont.) Patients who develop urticaria pigmentosa during early childhood tend to have only cutaneous involvement, and their disease will almost always involute over a period o years. Figure 20-58 illustrates a child with widespread lesions; one has spontaneously blistered due to the release o histamines.

Figure 20-59 shows the same process in child with skin o color. In general, no treatment is necessary, but it is important to provide the parents with a list o medications that might cause the sudden and potentially li e-threatening release o histamine. T ese include aspirin, codeine, morphine, polymyxin B, aminoglycosides, and NSAIDs. Immersion in a very hot bath may have the same e ect.

Fig ure 20-60

Fig ure 20-61

Bullous mastocytosis (Dif use cutaneous mastocytosis) T is disease orm is much less common than urticaria pigmentosa and much more serious. T e disease may be present at birth, or begin during in ancy. T e skin tends to be leathery and thickened as a result o widespread in ltration o mast cells in the dermis. Children su er rom severe recurrent episodes o urticaria and blistering.

As illustrated in Fig. 20-61, the blisters vary in size and may be hemorrhagic. T e occurrence o bullae may be accompanied by wheezing, diarrhea, hypotension, and shock-like symptoms. In such patients, management o the disease may present a signi cant challenge. In the newborn, di use cutaneous mastocytosis must be di erentiated rom other blistering disorders, including staphylococcal scalded skin syndrome (Figs. 3-7 through 3-11), and a skin biopsy is mandatory. Familial occurrence o bullous mastocytosis has been reported.

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Fig ure 20-62

Fig ure 20-63

Nevus lipomatosus super cialis T is is a hamartoma composed o at tissue situated in dermis. T e lesions are papillomatosis and have a yellowish hue. Nevus lipomatosus is not associated with any syndromes or underlying disorders, and can be excised i dis guring.

Leiomyoma T e lesions are f eshy papules or nodules. In some case, they are associated with intense pain. T ey have no malignant potential. Surgical removal is curative or solitary lesions but may be more complicated or numerous and widely spaced lesions such as those illustrated in Fig. 20-63.

Fig ure 20-64

Fig ure 20-65

Smooth muscle and pilar hamartoma T is lesion typically presents as a solitary rm plaque, most o en located on the trunk or proximal extremities and noted at birth or within the rst ew weeks o li e. T e lesions are f esh-colored to slightly erythematous initially, but eventually tend to develop a light brown color. here is usually increased hair growth within the lesion.

Histologically, the lesion is characterized by the presence o well-de ned bundles o smooth muscle. Firm stroking may elicit asciculations, a phenomenon noted in the lesion pictured in Fig. 20-65). T is is termed pseudo-Darier’s sign. T e term is based on a similarity to Darier sign: the swelling o a lesion o mastocytosis elicited also by stroking (Fig 20-55).

Se ction 2 0 . Dis orde rs o the De rm is (Inf ltrate s , Atrophie s , and Nodule s )

255

Fig ure 20-66

Fig ure 20-67

Lymphomatoid papulosis T is uncommon skin disorder is characterized by recurrent episodes o red to brown papules and nodules. Most common locations are the trunk and extremities, and the ace is usually spared. T e crops o lesions may last or several weeks, but larger nodules may be more persistent.

Ulceration is not uncommon, as illustrated in Figs. 20-67 and 20-68, and lesions may heal with hyperpigmentation, hypopigmentation, or true scarring.

Fig ure 20-68

Lymphomatoid papulosis T e prognosis o lymphomatoid papulosis is variable. In some patients, there is complete resolution a er a single episode, and, in others, recurrences occur or a period o years. In adult patients, there is a signi cant risk o developing some orm o malignant lymphoma. In children, the risk appears to be less, but there are reported cases o lymphomatoid papulosis developing into non-Hodgkin lymphoma.

SECTION

21 Drug Eruptions

258

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Fig ure 21-1

Fig ure 21-2

Drug eruptions Diagnosing drug eruptions has become a common experience to practitioners in all branches o modern medicine. T e pro usion o drugs now available, the continuous in ux o new drugs, and the capability o drugs to cause actions di erent rom or in addition to their pharmacologically desirable actions make adverse cutaneous reactions an inevitable act o modern medical practice. T e kinds o cutaneous reactions are varied. Illustrated in Fig. 21-1 is a reaction to amoxicillin. Eruptions rom amoxicillin are more requently seen in children with in ectious mononucleosis.

Morbili orm rashes are the most common orm o drug eruption. Illustrated in Fig. 21-2 is a reaction to chloroquine (Plaquenil). Other common causes are amoxicillin, cephalosporins, semisynthetic penicillins, and barbiturates. Constitutional symptoms o low-grade ever and malaise may be associated with such drug eruptions.

Fig ure 21-3

Fig ure 21-4

Drug eruptions Drug eruptions may be uncom ortably pruritic, but they are rarely serious and usually subside airly quickly upon elimination o the causative drug. Illustrated in Fig. 21-3 is reaction to a sul onamide.

Urticaria multiforme (also Figs. 16-21 to 16-23) T is condition, also known as acute annular urticaria, is a benign and airly common hypersensitivity reaction that presents with urticarial plaques and annular or arcuate urticarial lesions, along with acral edema. When the cause is a medication, the most likely culprits are amoxicillin, cephalosporins, and macrolides.

259

Se ction 2 1 . Drug Eruptions

Fig ure 21-5

Fig ure 21-6

Fixed drug eruption Another common type o adverse reaction to drugs is the so-called xed drug eruption. T e term f xed is intended to suggest that the cutaneous change, occurring or the rst time in given sites (anywhere), recurs in those same sites upon subsequent and repeated administration. Upon subsequent provocation, new reactions in new sites may occur, but original sites always are again.

T ere are a number o drugs that are well known or their propensity to cause xed drug eruptions. T e most common are the tetracyclines, sul onamides, and, NSAIDs, Other causes are aspirin, dapsone, and a variety o sedatives. T e clinical morphology o a xed drug eruption is usually a roundish plaque that is palpably edematous and purplish as shown in Fig. 21-6. Sometimes xed drug eruptions are bullous.

Fig ure 21-7

Fig ure 21-8

Fixed drug eruption Figure 21-7 illustrates xed drug eruption in a particularly common location. Proo o a drug as cause o an eruption is sometimes certain, on the basis o repeated experience, but of en conjectural or circumstantial, especially in the case o newly introduced drugs.

Figure 21-8 illustrates a larger lesion. T e erythema o acute in ammation and the residual pigmentary change rom a previous episode are seen simultaneously. T e area o dark skin does not always completely ade between episodes.

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We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 21-9

Fig ure 21-10

Hypersensitivity syndrome Each o the anticonvulsant medications phenytoin (Dilantin), phenobarbital, and carbamazepine ( egretol), as well as numerous other medications have been reported to cause this severe orm o drug reaction. T e mucocutaneous mani estations are strawberry tongue and a generalized scarlatini orm eruption that is ollowed by desquamation. T e rash may also be purpuric. T e presence o high ever, lymphadenopathy, and hepatosplenomegaly may give the alse impression o an in ectious disease. Elevations in blood urea nitrogen, liver enzymes, and peripheral eosinophil count may also be present.

Drug-induced gingival overgrowth Drug-induced gingival overgrowth can be caused by phenytoin, cyclosporine, and a number o calcium channel blockers. T ere is evidence that genetic predisposition, length o treatment with the above medications, and poor dental hygiene all contribute to the evolution o this disorder.

Fig ure 21-11

Fig ure 21-12

Iododerma and bromoderma Iodides and bromides are drugs that can cause severe adverse cutaneous reactions. T ese can be acnei orm, uruncular, carbuncular, chancri orm, pyodermatous, or granulomatous. Iodides and bromides are widely distributed, not only in oods and in the environment, but also in proprietary and ormally prescribed medications.

Figures 21-11 and 21-12 show adverse reactions rom the two halides. Lesions like those o acne or olliculitis caused by an iodide are shown in Fig. 21-11. Figure 21-12 shows a granulomatous reaction caused by a bromide.

261

Se ction 2 1 . Drug Eruptions

Fig ure 21-13

Fig ure 21-14

Acute generalized exanthematous pustulosis (AGEP) T is severe orm o drug eruption begins with high ever. Patients develop a generalized redness and edema, ollowed by numerous pustules that are sterile and do not necessarily arise rom hair ollicles. T e entire process resolves with generalized desquamation.

reatment consists o withdrawal o the causative medication (of en a beta-lactam or macrolide antibiotic), and, sometimes, systemic corticosteroids. Rarely, AGEP may be the result o an antecedent in ection and not a reaction to a drug.

SECTION

22 Panniculopathies

264

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 22-1

Fig ure 22-2

Subcutaneous fat necrosis of the newborn T is is a sel -resolving and benign condition that is seen in healthy newborns. T e etiology o this disorder is probably ischemic injury to subcutaneous at. Lesions o en develop at sites o pressure.

T e in ants develop single or multiple rm red-purple nodules or plaques that are asymptomatic. Cheeks, back, buttocks, and thighs are the most common locations. It is di cult to capture the quality o panniculitis in the gure, but a sense o it can be appreciated on the back o the patient pictured in Fig. 22-2.

Fig ure 22-3

Fig ure 22-4

Subcutaneous fat necrosis Lesions may be present at birth, or they may develop during the rst month o li e. Most lesions resolve spontaneously over a period o 2 to 4 weeks, but some last signi cantly longer. T ere is usually no residual atrophy or scarring. Subcutaneous at necrosis is occasionally associated with hypercalcemia, as was the case in this patient.

Sclerema neonatorum Unlike the condition just described, sclerema neonatorum presents itsel as symmetrical areas o induration on cheeks, shoulders, buttocks, and calves. T e skin over involved subcutaneous at is uni ormly board-like, cold, and livid in color, as though rozen. In ants so af ected appear rigid because mobility is inter ered with by the sclerema and they are severely ill. Mortality is high. T e condition is more common in premature in ants and in those with severe underlying disease, such as sepsis or dehydration.

265

Se ction 2 2 . Panniculopathie s

Fig ure 22-5

Fig ure 22-6

Erythema nodosum T is condition, characterized by red, tender, subcutaneous nodules on the extensor aspects o the legs between knees and ankles has numerous causes. T e most important conditions are streptococcal upper-respiratory in ections, ulcerative colitis, histoplasmosis, coccidioidomycosis, tuberculosis, syphilis, and leprosy.

Another condition that is sometimes revealed by investigation o erythema nodosum is sarcoidosis. Drugs, including oral contraceptives, appear to be the cause o particular cases o erythema nodosum. In many cases, however, no clear etiology can be ound.

Fig ure 22-7

Fig ure 22-8

Panniculitis from cold Local exposure to cold leads to the ormation o ice crystals within cells. Injury to cell contents occurs during both cooling and thawing. Cold panniculitis may occur in a child whose glove or boot has lled with snow. T e patient shown in Fig. 22-7 was out in reezing weather with a strap holding her hat.

Cold panniculitis can also occur in a youngster who has been sucking on a rozen dessert product. So-called “popsicle panniculitis” is illustrated in Fig. 22-8. T is condition is sel -limited and requires only symptomatic relie .

266

We inbe rg’s Color Atlas of Pe diatric De rm atology

Fig ure 22-9

Fig ure 22-10

Lipoatrophy (localized) A localized area o at atrophy may result rom the injection o insulin or corticosteroids. In most cases, the change in the subcutaneous tissue is temporary. In this patient, as sometimes occurs, there was no antecedent injection and the etiology was unknown.

Lipoatrophy secondary to reticular hemangioma A rare orm o hemangioma o in ancy presents as a macular lesion with numerous ne telangiectasias. T is lesion, now re erred to as reticular hemangioma, has also been termed abortive or minimalgrowth hemangioma. Hemangiomas o this type may be associated with a ocal area o cutaneous depression, as pictured in Fig. 22-10, or a larger band-like or segmental area o lipoatrophy.

Fig ure 22-11

Fig ure 22-12

Progressive partial lipodystrophy T is disorder is characterized by the loss o all adipose tissues in the upper portion o the body. Patients characteristically have the emaciated “sunken cheek” appearance that is seen in Fig. 22-11. A requent complication o progressive lipoatrophy is membranoproli erative glomerulonephritis, associated with decreased levels o the third component o complement. T e majority o patients are emale.

Acquired generalized lipodystrophy T is rare disorder develops during childhood and adolescence and is characterized by loss o subcutaneous at involving the ace, trunk, abdomen, and extremities. Figure 22-12 illustrates the prominence o veins and muscles in a child lacking subcutaneous at. Patients may develop diabetes, elevated serum triglycerides, and autoimmune thyroid disease.