Dermatology [4 ed.] 9780702062759, 9780702063428

Table of contents :
Cover
Front Matter
Copyright
Video Table of Contents
Preface
User Guide
Volumes, Sections and Color Coding
Volume One
Volume Two
Basic Science Chapters
Therapeutic Ladders
Dermatology Website
Dedication
Figures and Tables
Section 1: Overview of Basic Science
Chapter 0: Basic Principles of Dermatology
Abstract
Keywords:
Chapter Contents
Introduction to Clinical Dermatology
Etiologic Premises
Inflammatory versus neoplastic
Morphology
Palpation and appreciation of textural changes
Color
Variation in skin color within the human population
Configuration and Distribution
Configuration
Distribution
Augmented Examination – Wood’s Lamp and Dermoscopy
Temporal Course
The Role of Dermatopathology in Clinicopathologic Correlation
The Skin Biopsy
Site selection
Biopsy techniques
Handling of the specimen after biopsy
Classification of Inflammatory Skin Diseases by Pattern Analysis
Ten patterns defined
Perivascular dermatitis
Interface dermatitis
Spongiotic dermatitis
Psoriasiform dermatitis
Vesiculobullous and pustular dermatoses
Intraepidermal (see Fig. 0.14E)
Subepidermal vesiculation (see Fig. 0.14F)
Vasculitis/pseudovasculitis
Nodular and diffuse dermatitis
Folliculitis/perifolliculitis
Fibrosing/sclerosing conditions
Panniculitis
Invisible dermatoses
Deposition of Materials Within the Skin
Histologic Stains
Immunohistochemical Testing
Introduction to the Use of Dermoscopy (Dermatoscopy)
Conclusion
References
Chapter 1: Anatomy and Physiology
Abstract
Keywords:
Introduction
Structure and Function
Conventional Concepts of the Structure of Skin
Epidermis
Dermis
Dermal–epidermal interface
Knowledge of the Function and Structure of Skin Begins With Skin Disease
The Central Role of Protecting DNA as a Function of Skin
The requirements of skin are identified in its failings
Preventing Infection: Skin as an Immunologic Organ
Failure of immunity: infection
Warts
Dermatophytosis
Opportunistic infections in the setting of human immunodeficiency virus infection
Leprosy (Hansen disease)
Faulty immunity: autoimmunity
Failure of immunity: cancer
Maintaining a Barrier: Skin as a Protective Organ
Failure of protection against toxic chemicals
Failure to protect against dehydration and infection: toxic epidermal necrolysis
Failure to protect against UV radiation: albinism
Maintaining the Integrity of Skin: Repair Mechanisms
Failure to effectively repair injury
Delayed wound healing
Keloids
Xeroderma pigmentosum
Providing Circulation: Skin as a Nutritive Organ
Failure of circulation: arteries and veins
Embolic occlusion of arteries
Vasculitis
Occlusive vasculopathy
Venous insufficiency
Failure of circulation: lymphatic blockage
Interfacing With External and Internal Environments: Skin as a Communicating Organ
Abnormality of neurologic communication: excessive sensitivity
Abnormality of neurologic communication: excessive sweating
Abnormality of neurologic communication: decreased sensitivity
Communication via hormones and cytokines
Hormones
Cytokines
Cellular communication
Failure of cytokine and cellular communication
Regulating Temperature: Skin as a Thermoregulatory Organ
Failure of thermoregulation: effects of excessive heat
Heat injury as therapy
Failure of thermoregulation: effects of excessive cold
Cold injury as therapy
Interpersonal Communication: the Skin Conveys Beauty, Attracts Attention and Contributes to Self-Identity
Failures to present an attractive appearance
Pigmentary turmoil
Inappropriate hair distribution
Undesirable fat distribution
The next steps
An Important Corollary, With an Eye to the Future
Acknowledgment
References
Chapter 2: Skin Development and Maintenance
Abstract
Keywords:
Chapter Contents
Introduction
Embryonic Origin of the Skin
Epidermal Development
Clinical Relevance
Development of Specialized Cells Within the Epidermis
Clinical Relevance
Development of the Dermis and Subcutis
Clinical Relevance
Development of the Dermal–Epidermal Junction
Clinical Relevance
Development of Skin Appendages (Adnexae)
Hair Follicle Development
Nail Development
Eccrine and Apocrine Sweat Gland Development
Clinical Relevance
Skin Stem Cells
Epidermal Stem Cells
Hair Follicle Stem Cells
Stem Cell Plasticity
Stem Cell-Based Therapy for Genetic Skin Disease
Prenatal Diagnosis of Genodermatoses
Significance of Skin Development in Postnatal Life
References
Chapter 3: Molecular Biology
Abstract
Keywords:
Introduction
Experimental Techniques
Tissue Processing
The Foundations of Molecular Techniques for Analyzing DNA, RNA, and Protein
Measuring Spatial Distribution Within Tissues
Measuring the Transcriptome and Proteome
Genetically Engineered Mouse Models
Gene-Based Therapy for Skin Diseases
Conclusions
Copyright notice
References
Chapter 4: Immunology
Abstract
Keywords:
Introduction
Innate Immune Response
Complement
Toll-Like Receptors
Inflammasomes
Antimicrobial Peptides
Cytokines
Macrophages and Neutrophils
Eosinophils
Basophils and Mast Cells
Natural Killer Cells
Adaptive Immune Response
Antigen-Presenting Cells
Langerhans cells
Morphology of Langerhans cells
Ontogeny of Langerhans cells
Other dendritic cells
Antigen presentation
Antigen presentation cells: activation and migration
Antigen presentation to T cells
T Cells
T-cell development
T-cell receptor
T-cell receptor diversity
T-cell receptor signaling
Costimulatory signals
Clonal expansion
Effector functions of T cells
T helper cells
Cytotoxic T cells
Regulatory T cells
Natural killer T cells
γ/δ T cells
Dendritic epidermal T cells
Innate lymphoid cells
Lymphocyte recruitment
B Cells
Immunoglobulins
Immunoglobulin M
Immunoglobulin G
Immunoglobulin A
Immunoglobulin E
Immunoglobulin D
B-cell activation
Allergic Contact Hypersensitivity
Induction of CHS
Elicitation of CHS
Keratinocytes as immune targets and initiators
References
Section 2: Pruritus
Chapter 5: Cutaneous Neurophysiology
Abstract
Keywords:
Chapter Contents
Introduction
Pruritus Pathways
Central Pathways to Higher Nervous System Centers
Pruritus Receptor Units
Mediators of Pruritus
Histamine
Gastrin-Releasing Peptide
B-Type Natriuretic Peptide
Proteases
Opioid Peptides
Substance P
Neurotrophins
Prostanoids
Mediators That Activate Transient Receptor Potential Receptors
Other Peripheral Mediators of Itch
Other neurotransmitters
Peptidases
Other mediators with potential roles in itch
Immune Cells as Itch Mediators and Modulators
Chronic Itch
Peripheral Sensitization in Chronic Itch
Central Sensitization in Chronic Itch
Itch Related to Impaired Skin Barrier Function
Senescent Skin and Itch
Cholestatic Pruritus
Treatment of Pruritus
Pharmacologic Treatments
Behavioral Therapy
Future Directions
References
Chapter 6: Pruritus and Dysesthesia
Abstract
Keywords:
Chapter Contents
Introduction
Epidemiology
Evaluation of the Patient
History
Examination
Laboratory Investigation
Pruritus in Dermatologic Disease
Inflammatory Dermatoses
Urticaria
Atopic dermatitis
Psoriasis
Infestations
Scabies
Pediculosis (lice)
Cutaneous T-Cell Lymphoma (CTCL)
Dermatoses Induced by Pruritus-Associated Scratching or Rubbing
Prurigo Nodularis
Lichen Simplex Chronicus
Pruritus in Specific Locations
Scalp Pruritus
Anogenital Pruritus
Pruritus ani
Pruritus vulvae and scroti
Pruritus Variants
Aquagenic Pruritus
Pruritus in Scars
Post-Thermal Burn Pruritus
Fiberglass Dermatitis
Pruritus in Systemic Disease
Renal Pruritus
Cholestatic Pruritus
Hematologic Pruritus
Iron deficiency
Polycythemia vera
Pruritus and Malignancy
Hodgkin disease
Non-Hodgkin lymphoma
Leukemia
Endocrine Pruritus
Thyroid disease
Diabetes mellitus
Pruritus in HIV Infection and AIDS
Pruritus in Pregnancy
Pharmacologic Pruritus
Psychogenic Pruritus
Neurologic Etiologies of Pruritus and Dysesthesia
Sensory (Mono)Neuropathies With Pruritus and Dysesthesia
Notalgia paresthetica
Brachioradial pruritus
Meralgia paresthetica
Cheiralgia paresthetica
Digitalgia paresthetica
Regional Dysesthesias With Burning or Pain
Burning mouth syndrome
Burning scalp syndrome
Dysesthetic anogenital pain syndromes
Trigeminal neuralgia and trigeminal trophic syndrome
Complex regional pain syndrome (reflex sympathetic dystrophy)
Congenital Insensitivity to Pain and Related Conditions
Treatment
General Measures
Topical Treatment
Systemic Treatment
Physical Treatment Modalities
Psychological Approaches
References
Chapter 7: Psychocutaneous Diseases
Abstract
Keywords:
Introduction
Overview
Primary Psychiatric Disorders With Dermatologic Manifestations
Delusions of Parasitosis
Introduction
Clinical features
Epidemiology
Differential diagnosis
Management
General Features of Obsessive–Compulsive and Related Disorders
Body Dysmorphic Disorder
Introduction
Clinical features
Epidemiology
Management
Trichotillomania
Introduction
Clinical features
Epidemiology
Diagnosis and pathology
Differential diagnosis
Management
Excoriation Disorder
Introduction
Clinical features
Epidemiology
Differential diagnosis
Management
Acne Excoriée
Clinical features and epidemiology
Management
Dermatitis Artefacta
Introduction
Clinical features
Pathology
Epidemiology
Differential diagnosis
Management
Nonsuicidal Self-Injury
Clinical features and epidemiology
Management
Excessive Tanning Behavior
Secondary Psychiatric Disorders Resulting From Dermatologic Conditions
Somatic Symptom Disorder
Illness Anxiety Disorder
Treatment
References
Section 3: Papulosquamous and Eczematous Dermatoses
Chapter 8: Psoriasis
Abstract
Keywords:
Introduction
History
Epidemiology and Genetics
Genetic Factors
HLA studies
Genome-wide association studies
Functional genomic studies
Pathogenesis
Immunopathogenesis
Role of T cells and dendritic cells
Cytokines and chemokines
Innate immunity and role of keratinocytes
Triggering Factors
External triggering factors
Systemic triggering factors
Infections
HIV
Endocrine factors
Psychogenic stress
Drugs
Alcohol consumption, smoking and obesity
Clinical Features
Chronic Plaque Psoriasis
Guttate Psoriasis
Erythrodermic Psoriasis
Pustular Variants
Generalized pustular psoriasis
Pustulosis of the palms and soles
Acrodermatitis continua of Hallopeau
Special Locations
Scalp psoriasis
Flexural psoriasis
Oral mucosa
Nail psoriasis
Psoriatic Arthritis
Disorders Related to Psoriasis
Inflammatory linear verrucous epidermal nevus (ILVEN)
Reactive arthritis (formerly Reiter disease)
Sneddon–Wilkinson disease (subcorneal pustular dermatosis)
Associations Between Psoriasis and Other Diseases
Associations with skin diseases
Infections
Cancer
Association with internal diseases (including comorbidities)
Differential Diagnosis
Pathology
Papulosquamous Lesions
Initial lesion
Active lesion
Stable lesion
Pustular Psoriasis
Treatment
Topical Treatments
Corticosteroids
Vitamin D3 analogues
Anthralin
Topical retinoids
Additional topical treatments
Photo(chemo)therapy and Systemic Medications
Photo(chemo)therapy
Methotrexate
Cyclosporine
Systemic retinoids
Targeted immunomodulators (“biologic” therapies)
Apremilast and other systemic therapies
Therapeutic Management
General considerations
Management with topical agents
Management with photo(chemo)therapy and classic systemic medications
Targeted immunomodulators (“biologic” therapies)
How to combine treatments
Beneficial combinations
Contraindicated combinations and combinations with restricted use
Management of childhood psoriasis
Management of psoriasis in patients with comorbidities
Future Developments and Treatments
References
e-References
Chapter 9: Other Papulosquamous Disorders
Abstract
Keywords:
Chapter Contents
Small Plaque Parapsoriasis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Large Plaque Parapsoriasis
Introduction, History, and Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pityriasis Lichenoides et Varioliformis Acuta and Pityriasis Lichenoides Chronica
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pityriasis Rubra Pilaris
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pityriasis Rosea
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pityriasis Rotunda
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Granular Parakeratosis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 10: Erythroderma
Abstract
Keywords:
Introduction
Historical Perspective
Epidemiology
Pathogenesis
Clinical Features
Cutaneous Manifestations
Systemic Manifestations
Specific Findings of the Underlying Disease
Psoriasis
Atopic dermatitis
Drug reactions
Idiopathic erythroderma
Cutaneous T-cell lymphoma (Sézary syndrome and erythrodermic mycosis fungoides)
Pityriasis rubra pilaris
Papuloerythroderma of Ofuji
Paraneoplastic erythroderma
Bullous dermatoses
Ichthyoses
Staphylococcal scalded skin syndrome
Omenn syndrome
Pathology
Differential Diagnosis
Treatment
References
Chapter 11: Lichen Planus and Lichenoid Dermatoses
Abstract
Keywords:
Chapter Contents
Lichen Planus
Introduction
History
Epidemiology
Pathogenesis
Target antigens
Hepatitis C virus
Other viruses
Vaccines
Bacteria
Contact allergens
Drugs
Autoantigens, including tumor antigens
Effector cells
The innate immune system, regulatory T cells (Tregs), and Th17 cells
Effector T cells’ access to the epidermis
Sweating disturbance
Clinical Features
Actinic LP
Acute (exanthematous) LP
Annular LP
Atrophic LP
Bullous LP and LP pemphigoides
Hypertrophic LP
Inverse LP
LP pigmentosus
Lichen planopilaris
Linear LP
Discoid lupus erythematosus/lichen planus overlap syndrome
Nail LP
Oral LP
Ulcerative LP
Vulvovaginal LP
Lichenoid drug eruption (drug-induced LP)
Pathology
Differential Diagnosis
Treatment
Systemic therapies
Phototherapy
Lichen Striatus
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Lichen Nitidus
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Erythema Dyschromicum Perstans
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Keratosis Lichenoides Chronica
Actinic Lichen Nitidus (Summertime Actinic Lichenoid Eruption)
Annular Lichenoid Dermatitis (Of Youth)
References
Chapter 12: Atopic Dermatitis
Abstract
Keywords
Introduction
History and Definitions
Epidemiology
Pathogenesis
Genetic Factors
Epidermal Barrier Dysfunction
Filaggrin and other structural proteins
Stratum corneum lipids
Proteases and protease inhibitors
Immune Dysregulation
Thymic stromal lymphopoietin (TSLP)
IL-4 and IL-13
Other cytokines
Innate lymphoid cells
The Cutaneous Microbiome
Clinical Features
Disease Course
Regional Variants of Atopic Dermatitis
Associated Features
Pruritus
Atopic stigmata
Pityriasis alba
Complications
Infections
Ocular complications
Diagnostic Criteria
Pathology
Differential Diagnosis
Treatment
General Approach
Educational interventions
Bathing
Moisturizers
Topical Anti-Inflammatory Therapy
Topical corticosteroids
Topical calcineurin inhibitors
Crisaborole
Wet wrap therapy
Phototherapy
Systemic Anti-Inflammatory Therapy
Dupilumab
Cyclosporine
Azathioprine
Methotrexate
Mycophenolate mofetil
Systemic Corticosteroids
Adjunctive Therapy
Antimicrobials and antiseptics
Antihistamines
Omalizumab
Systemic immunotherapy
Dietary supplements
Management of Coexisting Allergic Disease
Food allergies
Aeroallergen reactivity
Allergic contact dermatitis
Complementary Therapies
Prevention
Probiotics/prebiotics
Emollient therapy as prevention
References
Chapter 13: Other Eczematous Eruptions
Abstract
Keywords:
Chapter Contents
Seborrheic Dermatitis
Introduction
History
Epidemiology
Pathogenesis
Malassezia
Malassezia and seborrheic dermatitis
Active sebaceous glands and seborrheic dermatitis
Immune responses to Malassezia in seborrheic dermatitis
Clinical Features
Infantile seborrheic dermatitis
Adult seborrheic dermatitis
Pathology
Differential Diagnosis
Treatment
Infantile seborrheic dermatitis
Adult seborrheic dermatitis
Asteatotic Eczema
Introduction
History and Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Disseminated Eczema (Autosensitization)
Introduction
History and Pathogenesis
Epidemiology
Clinical Features
Pathology
Differential Diagnosis
Treatment
Nummular Dermatitis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pityriasis Alba
HTLV-Associated Infective Dermatitis
Introduction and Pathogenesis
History and Epidemiology
Clinical Features
Differential Diagnosis
Treatment
Regional Eczematous Disorders
Stasis Dermatitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Dyshidrotic Eczema
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Infectious Eczematous Dermatitis
Juvenile Plantar Dermatosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Diaper Dermatitis
Epidemiology
Pathogenesis
Clinical features
Differential diagnosis
Treatment
References
Chapter 14: Allergic Contact Dermatitis
Abstract
Keywords:
Introduction
History of Patch Testing
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Patch Testing
Technique
Interpreting the test
Treatment and Patient Education
Allergens
Nickel
Methylisothiazolinone (MI)
Neomycin Sulfate
Fragrance Mix
Bacitracin
Myroxylon pereirae
Cobalt
Formaldehyde
Quaternium-15
p-Phenylenediamine
Thimerosal
Gold
Corticosteroids
Textile Dermatitis
Rubber Chemicals
Systemic Contact Dermatitis
Airborne Contact Dermatitis
References
Chapter 15: Irritant Contact Dermatitis
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Acute Irritant Contact Dermatitis
Acute Delayed Irritant Contact Dermatitis
Irritant Reaction Irritant Contact Dermatitis
Cumulative Irritant Contact Dermatitis
Asteatotic Dermatitis
Traumatic Irritant Contact Dermatitis
Pustular and Acneiform Irritant Contact Dermatitis
Non-erythematous Irritant Contact Dermatitis
Subjective or Sensory Irritant Contact Dermatitis
Airborne Irritant Contact Dermatitis
Frictional Irritant Contact Dermatitis
Contact Urticaria
Pathology
Classification of Irritant Chemicals
Acids
Alkalis
Metal salts
Solvents
Alcohols/glycols
Detergents and cleansers
Disinfectants
Plastics
Food and plants
Water
Bodily fluids
Differential Diagnosis
Prognosis
Treatment
References
Chapter 16: Occupational Dermatoses
Abstract
Keywords:
Introduction
History
Epidemiology
The Occupational Dermatoses
Contact Dermatitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Allergic contact dermatitis (see Ch. 14)
Irritant contact dermatitis (see Ch. 15)
Chemical burns
Fiberglass dermatitis
Phototoxic eruptions (see Ch. 87)
Mechanical
Pathology
Differential diagnosis
Treatment
Contact Urticaria
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Foods
Latex
Low-molecular-weight chemicals
Protein contact dermatitis
Pathology
Differential diagnosis
Treatment
Occupational Skin Cancer
Introduction
History
Epidemiology
Pathogenesis
Clinical features and diagnosis
Pathology and treatment
Occupational Acne
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Oil acne and tar acne
Acne mechanica
Acne cosmetica
Chloracne
Acne due to halogens
Pathology
Treatment
Vibration White Finger
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Infections
References
Chapter 17: Dermatoses Due to Plants
Abstract
Keywords:
Chapter Contents
Introduction
Botanical Aspects
Poison ivy – just as allergenic despite a name change
Binomial nomenclature
Plant identification
Patch Testing
Immunologic Contact Urticaria
Epidemiology
Pathogenesis (see Ch. 18)
Clinical Features
Differential Diagnosis and Pathology
Treatment
Toxin-Mediated (Non-Immunologic) Contact Urticaria
History
Epidemiology
Pathogenesis (see Ch. 18)
Clinical Features
Pathology (see Ch. 18)
Differential Diagnosis (see Ch. 18)
Treatment
Mechanical Irritant Dermatitis
Epidemiology
Pathogenesis
Clinical Features
Treatment
Chemical Irritant Dermatitis
History
Epidemiology
Pathogenesis, Clinical Features and Treatment (see Ch. 15)
Phytophotodermatitis
History
Epidemiology
Apiaceae
Rutaceae
Moraceae
Other families
Pathogenesis
Clinical Features
Phytophotoallergic Contact Dermatitis
Treatment
Allergic Contact Dermatitis (See Ch. 14)
Epidemiology
Anacardiaceae and related families
Toxicodendron identification
Allergenic Anacardiaceae
Cross-reactors from other families
Asteraceae and related families
Asteraceae identification
Allergenic Asteraceae
Cross-reactors from other families
Pathogenesis
Anacardiaceae allergens
Asteraceae allergens
Clinical Features
Anacardiaceae dermatitis
Asteraceae dermatitis
Photosensitivity and Asteraceae allergy
Treatment (Anacardiaceae and Asteraceae Dermatitis)
Other Plant Families Causing Allergic Contact Dermatitis
Alliaceae
Alstroemeriaceae and Liliaceae
Myrtaceae
Botanical products
Occupational Plant Dermatoses
Bakers
Florists and Horticulturalists
Food Handlers
Outdoor Workers
Dermatoses Erroneously Ascribed to Plants
Lichen Dermatitis
Chlorella Photodermatitis
Seaweed Dermatitis
Pseudo-Phytodermatitis From Plant Hitchhikers
References
Section 4: Urticarias, Erythemas and Purpuras
Chapter 18: Urticaria and Angioedema
Abstract
Keywords:
Introduction
Definition of Urticaria
Epidemiology
Pathogenesis
The Mast Cell
Distribution and diversity
Degranulating stimuli
Proinflammatory mediators
Blood Vessels
Blood
Autoantibodies
Leukocytes
Nerves
Mechanisms of Urticaria Formation
Mast cell-dependent urticaria
Mast cell-independent urticaria
C1 esterase inhibitor (C1 inh) deficiency
Clinical Features
Clinical Diversity
Classification
Acute Versus Chronic Urticaria
Spontaneous Urticaria
Associations
Inducible Urticarias (syn. Physical Urticarias)
Urticaria due to mechanical stimuli
Dermographism (literally “skin writing”) (syn. factitious urticaria)
Immediate dermographism
Rarer forms of dermographism
Delayed pressure urticaria
Vibratory angioedema
Urticaria due to temperature changes
Heat exposure
Heat contact urticaria
Cold exposure
Primary cold contact urticaria
Secondary cold contact urticaria
Reflex cold urticaria
Familial cold urticaria
Urticaria due to sweating or stress
Cholinergic urticaria
Exercise-induced anaphylaxis (EIA)
Adrenergic urticaria
Urticaria due to other exposures
Solar urticaria
Aquagenic urticaria
Contact Urticaria (see Ch. 16)
Food contact hypersensitivity syndrome
Angioedema Without Wheals
Drug reactions
Hereditary angioedema (HAE), types I and II (inherited C1 inh deficiency)
Hereditary angioedema (HAE), type III
Distinctive Syndromes With Urticarial Lesions
Hereditary periodic fever syndromes
Cryopyrin-associated periodic syndromes (CAPS)
Other syndromes
Acquired autoinflammatory syndromes
Schnitzler syndrome
Adult-onset Still disease
Episodic angioedema with eosinophilia (Gleich syndrome)
Systemic capillary leak syndrome
Urticarial Vasculitis (see Ch. 24)
Pathology
Diagnosis and Differential Diagnosis
Diagnosis
Acute urticaria
Chronic urticaria
Physical urticarias
Urticarial vasculitis
Angioedema without wheals
Treatment
Acute Urticaria, Chronic Spontaneous and Inducible Urticarias
First-line therapies
Classic antihistamines
Second-generation antihistamines
Addition of H2 antagonists to conventional H1 antihistamines
Antihistamines in pregnancy
Second-line therapies (targeted interventions)
Drug therapies
Non-drug therapies
Third-line therapies
C1 Esterase Inhibitor (C1 inh) Deficiency
Prognosis
References
Chapter 19: Figurate Erythemas
Abstract
Keywords:
Chapter Contents
Introduction
Erythema Annulare Centrifugum
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Erythema Marginatum
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Erythema Migrans
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Erythema Gyratum Repens
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 20: Erythema Multiforme, Stevens–Johnson Syndrome, and Toxic Epidermal Necrolysis
Abstract
Keywords:
Chapter Contents
Erythema Multiforme
Introduction
History
Epidemiology
Pathogenesis
Herpes simplex virus and Mycoplasma pneumoniae
Clinical Features
Elementary skin lesions (Fig. 20.1)
Distribution of skin lesions (topography)
Mucosal lesions
Systemic symptoms
Natural history
Pathology
Differential Diagnosis
Treatment
Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 21: Drug Reactions
Abstract
Keywords
Introduction
Epidemiology
Pathogenesis
Immunologically Mediated Drug Reactions
Genetic factors
Non-immunologic Mechanisms (see Table 21.3)
Overdose
Pharmacologic side effects
Cumulative toxicity
Delayed toxicity
Drug–drug interactions
Alterations in metabolism
Exacerbation of disease
Idiosyncratic With a Possible Immunologic Mechanism (see Table 21.3)
Diagnostic Features
Clinical Features
Urticaria, Angioedema, and Anaphylaxis
Urticaria (see Ch. 18 for details)
Angioedema (see Ch. 18 for details)
Anaphylaxis
Exanthematous Drug Eruptions
Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS)
Serum Sickness-Like Eruption
Vasculitis (see Ch. 24)
Neutrophilic Drug Eruptions
Acute generalized exanthematous pustulosis
Sweet syndrome (acute febrile neutrophilic dermatosis) (see Ch. 26)
Halogenoderma
Other Neutrophilic Eruptions
Symmetrical Drug-Related Intertriginous and Flexural Exanthema (SDRIFE)
Bullous Eruptions
Fixed drug eruption
Linear IgA bullous dermatosis
Drug-induced bullous pemphigoid
Drug-induced pemphigus
Stevens–Johnson syndrome and toxic epidermal necrolysis (see Ch. 20)
Photosensitivity (see Ch. 87)
Phototoxicity
Photoallergy
Reactions to Anti-Neoplastic Agents – Chemotherapy, Targeted Therapy and Immunotherapy
Chemotherapy
Alopecia
Stomatitis
Additional reactions
Targeted therapy
Immunotherapy
Drug-Induced Adverse Reactions Involving Hair and Mucosae
Hair
Mucosal ulcerations
Other Drug-Induced Cutaneous Reactions
Acneiform eruptions (including folliculitis)
Anticoagulant-induced skin necrosis
Granulomatous reactions
Drug reactions in HIV infection
Drug-induced lupus erythematosus (LE)
Lymphomatoid drug reaction (see Ch. 121)
Pigmentary changes
Drug-induced psoriasis
Additional uncommon drug reactions
Cutaneous Side Effects of Vaccines and Injected Medications
Vaccine-induced reactions
Localized reactions to injected medications
Treatment
References
Chapter 22: Purpura
Abstract
Keywords:
Purpura
Introduction
The Time Course of Purpura
Coagulation
Overlap of Coagulation and Inflammatory Vessel Disease
Tests for Coagulation
Antiplatelet and Anticoagulant Agents
Cutaneous Surgical Procedures in Patients Receiving Antiplatelet/Anticoagulant Agents
Selected Purpura Syndromes
Pigmented Purpuric Dermatoses
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Hypergammaglobulinemic Purpura of Waldenström
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Gardner–Diamond Syndrome
Introduction
Clinical features
Pathology
Differential diagnosis and treatment
Mondor Syndrome of Superficial Thrombophlebitis
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
References
Chapter 23: Cutaneous Manifestations of Microvascular Occlusion Syndromes
Abstract
Keywords:
Chapter Contents
Introduction
Disorders of Platelet-Related Thrombopathy
Heparin-Induced Thrombocytopenia Syndrome
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Myeloproliferative Neoplasms
Introduction
Pathogenesis
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Paroxysmal Nocturnal Hemoglobinuria
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Primary Thrombotic Microangiopathy
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Disorders of Cryoprecipitation or Cryoagglutination
Introduction
Pathogenesis
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Disorders of Occlusion by Opportunistic Organisms Proliferating Within Vessels
Introduction/pathogenesis
Clinical features/pathology
Differential diagnosis
Treatment
Disorders of Occlusion by Emboli
Cholesterol Embolus
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Oxalate Embolus
Introduction
Clinical features
Pathology
Differential diagnosis
Treatment
Other Embolus-Associated Syndromes
Systemic Coagulopathies With Cutaneous Manifestations
Protein C and S Disorders, Congenital and Acquired
Introduction
Pathogenesis/clinical features
Neonatal purpura fulminans
Warfarin necrosis
Warfarin-associated venous limb gangrene
Purpura fulminans with sepsis
Purpura fulminans postinfection
Antiphospholipid Antibody/Lupus Anticoagulant Syndrome
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology and laboratory
Differential diagnosis
Treatment
Disorders of Vascular Coagulopathies With Cutaneous Manifestations
Sneddon Syndrome
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Livedoid Vasculopathy
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Malignant Atrophic Papulosis
Introduction
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Disorders of Cell-Related Vascular Occlusion
Red Blood Cell Occlusion
Intravascular Cellular Occlusion
Disorders of Occlusion, Miscellaneous
Cutaneous Calciphylaxis
Brown Recluse Spider (Loxosceles) Bite
Hydroxyurea-Associated Vascular Occlusion
Interferon-Associated Cutaneous Necrosis
Hematoma-Associated Retiform Purpura
References
Chapter 24: Cutaneous Vasculitis
Abstract
Keywords:
Chapter Contents
Introduction
Classification
Epidemiology
Pathogenesis
General Clinical Features
Pathology
Differential Diagnosis
Cutaneous Small Vessel Vasculitis
Introduction
Epidemiology
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Henoch–Schönlein Purpura (and IgA Vasculitis in Adults)
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Acute Hemorrhagic Edema of Infancy
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Urticarial Vasculitis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Erythema Elevatum Diutinum
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Cryoglobulinemic Vasculitis (Due to Mixed Cryoglobulinemia)
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Predominantly Small and Medium-Sized Vessel Vasculitides
ANCA-Associated Vasculitides
Introduction
Epidemiology
Pathogenesis
Natural history, clinical features, and treatment
Microscopic Polyangiitis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Granulomatosis With Polyangiitis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Eosinophilic Granulomatosis With Polyangiitis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Predominantly Medium-Sized Vessel Vasculitis
Polyarteritis Nodosa
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Temporal Arteritis
Diagnostic Approach to Patients With Vasculitis
History and Physical Examination
Laboratory Evaluation
Treatment
References
e-References
Chapter 25: Eosinophil-Associated Dermatoses
Abstract
Keywords:
Chapter Contents
Eosinophil Biology
Granular Contents
Tissue Effects and Innate Immunity
Eosinophil-Associated Dermatoses
Granuloma Faciale
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Papuloerythroderma of Ofuji
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Wells Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Hypereosinophilic Syndromes
Introduction
History
Epidemiology
Pathogenesis
Secondary (reactive) HES
Primary (neoplastic) HES
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 26: Neutrophilic Dermatoses
Abstract
Keywords:
Neutrophil Biology
Inflammation
Sweet Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Associated Diseases
Pathology
Differential Diagnosis
Treatment
Pyoderma Gangrenosum
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Associated Diseases
Pathology
Differential Diagnosis
Treatment
Behçet Disease
Introduction
History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Mucocutaneous involvement
Systemic involvement
Pathology
Diagnosis
Treatment
Bowel-Associated Dermatosis–Arthritis Syndrome
Introduction
History
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Copyright notice
References
Chapter 27: Pregnancy Dermatoses
Abstract
Keywords:
Chapter Contents
Pemphigoid Gestationis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Polymorphic Eruption of Pregnancy
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Intrahepatic Cholestasis of Pregnancy
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Atopic Eruption of Pregnancy
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Approach to the Pregnant Woman With Pruritus
Physiologic Changes During Pregnancy
Dermatoses Influenced by Pregnancy
Autoimmune Progesterone Dermatitis
References
Section 5: Vesiculobullous Diseases
Chapter 28: The Biology of the Basement Membrane
Abstract
Keywords:
Chapter Contents
Introduction
Origin of the Epidermal Basement Membrane
Basal Keratinocytes: Hemidesmosome-Anchoring Filaments and Other Integrin Complexes
Plectin
Bullous Pemphigoid Antigen 1
Bullous Pemphigoid Antigen 2
Integrins
Hemidesmosome-associated integrin α6β4
Plasma membrane-associated integrins
Tetraspan CD151
The Lamina Densa
Laminins
Type IV Collagen
Nidogen
Heparan Sulfate Proteoglycans
Anchoring Fibrils and the Sublamina Densa Region
Type VII Collagen
Microfibrils
Microthread-Like Fibers
Basement Membrane Remodeling
The Epidermal Basement Membrane in Bullous Diseases
Autoimmune Subepidermal Blistering Diseases
Inherited Subepidermal Blistering Diseases
Summary
References
Chapter 29: Pemphigus
Abstract
Keywords:
Introduction
History
Epidemiology
Fogo Selvagem
Pathogenesis
Pathogenic Autoantibodies in Pemphigus
Desmogleins as Pemphigus Antigens
Desmoglein Compensation Theory as Explanation for Localization of Blisters
Humoral and Cellular Autoimmunity in Paraneoplastic Pemphigus
Immunologic Mechanism of Pathogenic Autoantibody Production in Pemphigus
Clinical Features
Pemphigus Vulgaris
Pemphigus Vegetans
Pemphigus Foliaceus
Pemphigus Erythematosus (Senear –Usher Syndrome)
Herpetiform Pemphigus
Drug-Induced Pemphigus
Paraneoplastic Pemphigus
IgA Pemphigus
Pathology
Pemphigus Vulgaris
Pemphigus Foliaceus
Paraneoplastic Pemphigus
IgA Pemphigus
Differential Diagnosis
Differential Diagnosis of Pemphigus Vulgaris
Differential Diagnosis of Pemphigus Foliaceus
Differential Diagnosis of Paraneoplastic Pemphigus
Differential Diagnosis of IgA Pemphigus
Treatment
Pemphigus Vulgaris
Pemphigus Foliaceus
Paraneoplastic Pemphigus
IgA Pemphigus
References
Chapter 30: Pemphigoid Group
Abstract
Keywords:
Chapter Contents
Bullous Pemphigoid
Introduction
History
Epidemiology
Pathogenesis
Humoral and cellular responses
Clinical Features
Non-bullous pemphigoid
Bullous phase
Clinical variants
Associated Diseases
Drug-induced bullous pemphigoid
Diagnosis
Light microscopy
Direct immunofluorescence (DIF) microscopy
Indirect immunofluorescence (IIF) microscopy
ELISA
Other immunopathological studies
Differential Diagnosis
Prognosis
Monitoring
Treatment
Mucous Membrane (Cicatricial) Pemphigoid
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Mucous membranes
Cutaneous lesions
Diagnosis and Differential Diagnosis
Light microscopy and electron microscopy
Direct immunofluorescence (DIF) microscopy
Immunoelectron microscopy
Indirect immunofluorescence (IIF) microscopy
Immunochemical studies, including ELISA
Differential diagnosis
Prognosis
Treatment
Epidermolysis Bullosa Acquisita
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Diagnosis and Differential Diagnosis
Light microscopy and electron microscopy
Direct immunofluorescence (DIF) microscopy
Indirect immunofluorescence (IIF) microscopy
Immunoelectron microscopy
Other immunochemical studies, including ELISA
Differential diagnosis
Treatment
References
Chapter 31: Dermatitis Herpetiformis and Linear IgA Bullous Dermatosis
Abstract
Keywords:
Chapter Contents
Dermatitis Herpetiformis
Introduction
History
Epidemiology
Pathogenesis
Genetic predisposition
Gluten-sensitive enteropathy
Circulating antibodies
Granular IgA deposition
Iodide and dapsone
Associated Disorders and Malignancies
Clinical Features
Pathology
Laboratory evaluation
Differential Diagnosis
Treatment
Patient Support
Linear IgA Bullous Dermatosis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 32: Epidermolysis Bullosa
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
EB Simplex
Junctional EB
Dystrophic EB
Clinical Features
Cutaneous Findings
Extracutaneous Findings
Cutaneous Malignancies
Approach to Diagnosis
Differential Diagnosis
Treatment
Kindler Syndrome
Introduction
History
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 33: Other Vesiculobullous Diseases
Abstract
Keywords:
Chapter Contents
Bullosis Diabeticorum
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Coma Blisters
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Friction Blisters
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Bullous Small Vessel Vasculitis
Bullous Drug Eruptions
Bullous Insect Bite Reactions
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Delayed Postburn/Postgraft Blisters
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Edema Blisters
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Additional Vesiculobullous Disorders
References
Chapter 34: Vesiculopustular and Erosive Disorders in Newborns and Infants
Abstract
Keywords:
Common Causes
Erythema Toxicum Neonatorum
Transient Neonatal Pustular Melanosis
Sterile Transient Neonatal Pustulosis
Miliaria
Neonatal Cephalic Pustulosis
Cutaneous Candidiasis
Sucking Blisters
Exogenous Causes of Erosions
Uncommon and Rare Causes
Acropustulosis of Infancy
Eosinophilic Pustular Folliculitis of Infancy
Congenital and Neonatal Langerhans Cell Histiocytosis
Incontinentia Pigmenti
Autosomal Dominant Hyperimmunoglobulin E (Hyper-IgE) Syndrome
Very Rare Disorders
Neonatal Behçet Disease
Vesiculopustular Eruption in Transient Myeloproliferative Disorder of Down Syndrome
Congenital Erosive and Vesicular Dermatosis
Pyoderma Gangrenosum
Restrictive Dermopathy
Noma Neonatorum
Perinatal Gangrene of the Buttock
References
Section 6: Adnexal Diseases
Chapter 35: Structure and Function of Eccrine, Apocrine and Sebaceous Glands
Abstract
Keywords:
Chapter Contents
Eccrine, Apocrine, and Apoeccrine Sweat Glands
Introduction
Structure, Function and Pathophysiology
Eccrine Sweat Glands
Structure
Development
Function
Pathophysiology
Hyper- and hypohidrosis
Drug-related eccrine disorders
Disorders of sweat retention
Apocrine Sweat Glands
Structure
Development
Function
Pathophysiology
Apoeccrine Sweat Glands
Sebaceous Glands
Introduction
Structure, Function and Pathophysiology
Structure
Development
Function
Pathophysiology
References
Chapter 36: Acne Vulgaris
Abstract
Keywords:
Introduction
History
Epidemiology, Including Genetic and Dietary Factors
Genetic Factors
Dietary Factors
Pathogenesis
Follicular Hyperkeratinization
Hormonal Influences on Sebum Production and Composition
Inflammation in Acne
Propionibacterium acnes and the Innate Immune System
Clinical Features
Acne Variants
Post-adolescent acne in women
Acne fulminans
Acne conglobata and associated conditions
Solid facial edema
Neonatal acne (neonatal cephalic pustulosis)
Infantile acne
Mid-childhood acne
Preadolescent acne
Acne excoriée
Acne associated with endocrinologic abnormalities
Acne associated with genetic syndromes
Acneiform Eruptions
Drug-induced acne
Occupational acne, acne cosmetica, and pomade acne
Chloracne
Acne mechanica
Tropical acne
Radiation acne
“Pseudoacne” of the nasal crease
Idiopathic facial aseptic granuloma
Childhood flexural comedones
Pathology
Differential Diagnosis
Treatment
Topical Treatments
Topical retinoids
Benzoyl peroxide and other topical antibacterial agents
Other topical medications
Oral Treatments
Antibiotics
Hormonal therapy
Isotretinoin
Surgical Treatment
References
Chapter 37: Rosacea and Related Disorders
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Treatment
Rosacea-Like Disorders
References
Chapter 38: Folliculitis and Other Follicular Disorders
Abstract
Keywords:
Chapter Contents
Introduction
Superficial Folliculitis
Introduction
Clinical features
Pathology
Differential diagnosis
Treatment
Eosinophilic Folliculitis
Eosinophilic pustular folliculitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Immunosuppression-associated eosinophilic pustular folliculitis
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Eosinophilic pustular folliculitis of infancy
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Disseminate and Recurrent Infundibulofolliculitis
Disorders of Follicular Keratinization
Erythromelanosis Follicularis Faciei
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Keratosis Pilaris Atrophicans
Lichen Spinulosus
Introduction, history and epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Phrynoderma
Deep Folliculitis
Pseudofolliculitis Barbae
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Acne Keloidalis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Follicular Occlusion Tetrad
Hidradenitis Suppurativa
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Trichostasis Spinulosa
Viral-Associated Trichodysplasia
Acknowledgment
References
Chapter 39: Diseases of the Eccrine and Apocrine Sweat Glands
Abstract
Keywords:
Chapter Contents
Sweat Glands
Eccrine Sweat Glands
Apocrine Sweat Glands
Hyperhidrosis
Primary Hyperhidrosis
Secondary Hyperhidrosis
Disorders associated with cortical (emotional) hyperhidrosis
Hypothalamic (thermoregulatory) hyperhidrosis
Medullary (gustatory) hyperhidrosis
Physiologic medullary hyperhidrosis
Pathologic medullary hyperhidrosis
Spinal (cord transection) sweating
Autonomic dysreflexia
Compensatory hyperhidrosis
Non-neural hyperhidrosis
Diagnosis of Hyperhidrosis
Treatment of Hyperhidrosis
Hypohidrosis and Anhidrosis
Central and Neuropathic Hypohidrosis
Peripheral Anhidrosis
Diagnosis of Anhidrosis
Treatment of Anhidrosis
Abnormalities and Alterations of Eccrine and Apocrine Sweat Composition
Diagnostic Microscopic Changes in Eccrine Sweat Glands
Influence of Sweat on Skin Diseases
Additional Disorders of the Eccrine Sweat Gland
Miliaria
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Multiple Sweat Gland Abscesses
Neutrophilic Eccrine Hidradenitis
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Idiopathic Palmoplantar Hidradenitis
Granulosis Rubra Nasi
Keratolysis Exfoliativa
Grover Disease
Disorders of the Apocrine Sweat Gland
Fox–Fordyce Disease
Epidemiology and pathogenesis
Clinical
Pathology
Treatment
References
Section 7: Rheumatologic Dermatology
Chapter 40: Autoantibodies Encountered in Patients with Autoimmune Connective Tissue Diseases
Abstract
Keywords
Chapter Contents
Introduction
Chapter Organization
Historical Perspective
FANA: the Classic ANA Assay
Importance of Technical Aspects of the ANA Assay
“Normal” Versus “Abnormal” ANA Values
Clinical Significance of ANA
Immunofluorescence Patterns
Concept of Clinical Utility as Applied to aAb Testing
Clinical Value of aAb in Diagnosis Versus Disease Activity Monitoring
“Sontheimer’s Corollary” to Greenwald’s Law of Lupus
Phototherapy and the ANA Assay
How Does One Evaluate a Positive ANA Result in a Patient Suspected of Having SLE?
Autoantibodies Encountered in Lupus Erythematosus
Drug-Induced ANA/SLE
Induction of ANA and Anti-dsDNA Antibodies by Tumor Necrosis Factor Inhibitors
Autoantibodies Encountered in Idiopathic Inflammatory Dermatomyopathies
TIF1-γ (p155) and MDA5/CADM-140/RIG-I-like receptor IFIH1
Jo-1 and Mi-2
Autoantibodies Encountered in Systemic Sclerosis (Scleroderma) and Morphea (Localized Scleroderma)
Autoantibodies Encountered in Other Rheumatic Disorders That Can Affect the Skin
References
Chapter 41: Lupus Erythematosus
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Cutaneous Lupus – Three Major Forms
Classification
Discoid lupus erythematosus
Subacute cutaneous lupus erythematosus
Acute cutaneous lupus erythematosus (ACLE)
Cutaneous Lupus – Additional Variants (See Fig. 41.2)
Lupus erythematosus tumidus
Lupus panniculitis
Chilblain lupus
Discoid lupus erythematosus/lichen planus overlap syndrome
Neonatal lupus erythematosus (NLE)
Bullous lesions
Systemic Lupus Erythematosus
Nonspecific Cutaneous Lesions
Pathology
Histopathology
Antibody Deposits in Lesional Skin
Antibody Deposits Within Normal-Appearing Skin
Differential Diagnosis
Treatment
Topical Therapy
Systemic Therapy
Adjunctive Therapy
References
Chapter 42: Dermatomyositis
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Cutaneous Disease
Systemic Disease
Malignancy
Pathology
Differential Diagnosis and Evaluation
Treatment
References
Chapter 43: Systemic Sclerosis (Scleroderma) and Related Disorders
Abstract
Keywords:
Chapter Contents
Systemic Sclerosis
Introduction
History
Epidemiology
Pathogenesis
Vascular dysregulation
Immune dysregulation
Extracellular matrix dysregulation
Clinical Features
Classification and diagnostic criteria
Cutaneous features
Extracutaneous features
Autoantibodies
Pathology
Differential Diagnosis
Treatment
Raynaud phenomenon
Cutaneous ulcers
Cutaneous sclerosis
Other cutaneous complications
Internal organ involvement
Eosinophilic Fasciitis
History
Clinical Features
Pathology
Treatment
Nephrogenic Systemic Fibrosis
History
Epidemiology and Pathogenesis
Clinical Features
Pathology
Treatment
Stiff Skin Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Treatment
Sclerodermoid Syndromes Induced by Exogenous Substances
Toxic Oil Syndrome
Eosinophilia–Myalgia Syndrome
Sclerodermoid Syndromes Induced by Drugs
Sclerodermoid Syndromes Induced by Chemicals
Silicosis
References
Chapter 44: Morphea and Lichen Sclerosus
Abstract
Keywords:
Morphea
Introduction
History
Epidemiology
Pathogenesis
Vascular Changes
Control of Fibroblast Function by T-Cell-Derived Cytokines
Animal Model and Genetics
Triggering Events
Clinical Features
Plaque-Type Morphea
Variants
Linear Morphea and Parry–Romberg Syndrome
Generalized Morphea
Morphea in Childhood
Laboratory Findings
Pathology
Morpheaform (and Sclerodermoid) Inflammatory Syndromes
Differential Diagnosis
Lichen Sclerosus
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment of Morphea and Lichen Sclerosus
Phototherapies
Topical Therapies
Corticosteroids
Calcineurin inhibitors and imiquimod
Vitamin derivatives
Hormones
Systemic Treatments
Immunosuppression
Penicillin and its derivatives
Vitamin derivatives
Cytokines, TNF-α inhibitors, multi-kinase inhibitors
Surgery
Physical Therapy
References
Chapter 45: Other Rheumatologic Disorders and Autoinflammatory Diseases
Abstract
Keywords:
Chapter Contents
Systemic-Onset Juvenile Idiopathic Arthritis (Still Disease)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Adult-Onset Still Disease
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Relapsing Polychondritis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Sjögren Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Laboratory Findings
Pathology
Differential Diagnosis
Treatment
Mixed Connective Tissue Disease
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Extra-Articular Manifestations of Rheumatoid Arthritis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Rheumatoid nodules
Rheumatoid vasculitis
Bywaters lesions
Felty syndrome
Neutrophilic dermatoses
Other
Pathology
Differential Diagnosis
Treatment
Autoinflammatory Diseases
Acknowledgment
References
Section 8: Metabolic and Systemic Diseases
Chapter 46: Mucinoses
Abstract
Keywords:
Introduction
Classification
Primary Degenerative–Inflammatory Mucinoses
Dermal Mucinoses
Scleromyxedema
Introduction and definition
History
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lichen myxedematosus (localized variants)
Introduction
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Self-healing cutaneous mucinosis
Scleredema
Introduction
History
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Mucinoses associated with altered thyroid function
Localized (pretibial) myxedema
Definition
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Generalized myxedema
Definition
Pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Reticular erythematous mucinosis
Introduction and definition
History
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Papulonodular mucinosis associated with autoimmune connective tissue diseases
Treatment
Cutaneous focal mucinosis
Miscellaneous mucinoses
Primary Follicular Mucinoses
Follicular mucinosis
History, epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Urticaria-like follicular mucinosis
Primary Hamartomatous–Neoplastic Mucinoses
Mucinous Nevus
Superficial (Angio)Myxoma
References
Chapter 47: Amyloidosis
Abstract
Keywords:
Chapter Contents
Introduction
History
Epidemiology
Classification
Pathogenesis
Amyloid properties
Primary (Localized) Cutaneous Amyloidosis
Clinical features
Pathology
Differential diagnosis
Treatment
Secondary Cutaneous Amyloidosis
Systemic Amyloidosis
Primary Systemic Amyloidosis
Clinical features
Pathology
Differential diagnosis
Treatment
Secondary Systemic Amyloidosis
Hemodialysis-Associated Amyloidosis
Inherited Amyloidoses
Familial amyloidosis, Finnish type
Muckle–Wells syndrome
Familial Mediterranean fever
Sipple syndrome or multiple endocrine neoplasia (MEN) type 2A
Hypotrichosis simplex of the scalp
X-Linked Reticulate Pigmentary Disorder (Partington Amyloidosis)
Appendix
References
Chapter 48: Deposition Diseases
Abstract
Keywords:
Chapter Contents
Introduction
Gout
Introduction
Epidemiology
Pathogenesis
Clinical Features
Acute gouty arthritis
Chronic tophaceous gout
Pathology
Differential Diagnosis
Treatment
Pseudogout
Lipoid Proteinosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Colloid Milium
Introduction and Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Mucopolysaccharidoses
Clinical Features and Pathology
Treatment
References
Chapter 49: Porphyria
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Cutaneous Findings
Acute Porphyric Attack
Clinical and Laboratory Investigation
The Non-Acute Porphyrias
Porphyria Cutanea Tarda (PCT)
Erythropoietic Protoporphyria (EPP)
X-linked Dominant Protoporphyria (XLDPP)
Congenital Erythropoietic Porphyria (CEP)
Hepatoerythropoietic Porphyria (HEP)
Pseudoporphyria
Differential Diagnosis
Treatment
The Acute Porphyrias
Acute Intermittent Porphyria (AIP)
Variegate Porphyria (VP)
Hereditary Coproporphyria (HCP)
δ-Aminolevulinic Acid Dehydratase (ALA-D) Deficiency Porphyria
Differential Diagnosis
Treatment
Cutaneous symptoms
Acute porphyric attack
Appendix
References
Chapter 50: Calcifying and Ossifying Disorders of the Skin
Abstract
Keywords:
Chapter Contents
Introduction
Cutaneous Calcification
Dystrophic Calcification
Autoimmune Connective Tissue Disease
Treatment of dystrophic calcification
Panniculitis
Genetic Disorders
Infections
Neoplasms
Other
Metastatic Calcification
Renal Disease
Milk–Alkali Syndrome
Hypervitaminosis D
Mixed Calcification
Calciphylaxis
Treatment of calciphylaxis
Idiopathic Calcification
Idiopathic Calcified Nodules of the Scrotum
Subepidermal Calcified Nodule
Tumoral Calcinosis
Milia-like Calcinosis
Iatrogenic Calcification
Cutaneous Ossification (Osteoma Cutis)
“Secondary” Cutaneous Ossification
Laboratory Evaluation of Patients With Cutaneous Calcification and Ossification
References
Chapter 51: Nutritional Diseases
Abstract
Keywords:
Introduction
Epidemiology and Pathogenesis
Clinical Features
Protein–Energy Malnutrition
Essential Fatty Acid Deficiency
Vitamins and Trace Elements
Vitamins
Vitamin excess
Vitamin D
Trace elements
Zinc
Copper
Selenium
Anorexia Nervosa and Bulimia (Nervosa)
Obesity
Bariatric Surgery and Gastrointestinal Malabsorption
Pathology
Differential Diagnosis
Treatment
References
Chapter 52: Graft-versus-Host Disease
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Acute GVHD
Chronic GVHD
Pathology
Differential Diagnosis
Treatment
Acute GVHD
Chronic GVHD
References
Chapter 53: Dermatologic Manifestations in Patients with Systemic Disease
Abstract
Keywords:
Chapter Contents
Cutaneous Rheumatology
Cutaneous Hematology and Oncology
Cutaneous Endocrinology and Metabolic Disease
Cutaneous Gastroenterology
Other Systemic Diseases With Cutaneous Manifestations
References
Section 9: Genodermatoses
Chapter 54: Basic Principles of Genetics
Abstract
Keywords:
Introduction
Genetic Diseases
Mendelian Patterns of Inheritance
Autosomal Dominant Inheritance
Autosomal Recessive Inheritance
X-Linked Recessive Inheritance
X-Linked Dominant Inheritance
Exceptions to Basic Mendelian Patterns of Heritance
Epigenetics and Imprinting
Chromosomal Disorders
Complex Genetic Traits
Identification of Disease Genes
Linkage Analysis and Related Approaches
Refinement of the Linkage Interval
Positional Candidate Approach
Identification and Validation of Mutations
Massively parallel (Next-Generation) Sequencing
Genome-Wide Association Studies (GWAS)
Functional Genomics
References
Chapter 55: Genetic Basis of Cutaneous Diseases
Abstract
Keywords
Introduction
Mckusick’s Mendelian Inheritance in MAN
Genotype–Phenotype Correlations
Molecular Classification of Hereditary Skin Disorders
Keratin Defects
Defects in Intercellular Junctions
Desmosomal defects
Connexin defects
Defects in Keratinocyte–Extracellular Matrix Adhesion
Transmembrane Transporter Defects
Calcium pump defects
ATP-binding cassette transporter defects
Nuclear Envelope Defects
Defects in Pyrin/NOD Family Members and Related Proteins
Defects in DNA Repair Genes, Tumor Suppressor Genes and Oncogenes
Defective DNA repair and protection of genomic integrity
Defective tumor suppressor genes
Activated oncogenes
Defects in WNT/β-Catenin Signaling
Contiguous Gene Syndromes
Cutaneous Mosaicism
Revertant Mosaicism
Chromosomal Disorders
Insight Into Acquired Skin Disorders Provided by the Study of Genodermatoses
Prenatal and Preimplantation Genetic Diagnosis
Treatment for Hereditary Skin Disorders
Gene Therapy
Cell Therapy
Therapy Targeting Affected Molecular Pathways
References
Chapter 56: Biology of Keratinocytes
Abstract
Keywords:
Chapter Contents
Introduction
The Epidermis: Structure and Function
Keratin Intermediate Filaments
Keratins and Signaling
Hair Keratins
Epidermal Differentiation
Regulatory Pathways Involved in Epidermal Development and Differentiation
Genes required for establishing and maintaining basal keratinocytes
Genes required for terminal differentiation in mature epidermis
Ca2+ in epidermal differentiation
Genes required for terminal differentiation in embryonic epidermis
Keratinocyte Adhesion
Desmosomes
What happens when desmosomes do not function properly?
Other Types of Cell Junctions
Adherens junctions
Tight junctions
Keratinocyte–Matrix Interactions
Related Diseases
Keratin Disorders
Epidermolysis bullosa simplex
Epidermolytic ichthyosis and palmoplantar keratoderma
White sponge nevus of Cannon
Gastrointestinal disorders
Filaggrin Deficiency Disorders
Mouse Models for Structural and Desmosomal Proteins
Conclusion
References
Chapter 57: Ichthyoses, Erythrokeratodermas, and Related Disorders
Abstract
Keywords
Chapter Contents
Introduction
Ichthyoses
Ichthyosis Vulgaris
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Steroid Sulfatase Deficiency
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Epidermolytic Ichthyosis
History
Epidemiology
Pathogenesis
Clinical Features
Epidermolytic epidermal nevi (mosaic epidermolytic ichthyosis)
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Superficial Epidermolytic Ichthyosis
History
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Ichthyosis With Confetti
Ichthyosis Hystrix Curth–Macklin
History
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Ichthyosis Hystrix
Collodion Baby
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Lamellar Ichthyosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Congenital Ichthyosiform Erythroderma
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Harlequin Ichthyosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Netherton Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Sjögren–Larsson Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Neutral Lipid Storage Disease With Ichthyosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Trichothiodystrophy With Ichthyosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Other Ichthyoses and Related Disorders
Erythrokeratodermas
Erythrokeratodermia Variabilis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Progressive Symmetric Erythrokeratoderma (PSEK)
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Keratitis–Ichthyosis–Deafness Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
X-Linked Dominant Ichthyosiform Disorders
CHILD Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
Conradi–Hünermann–Happle Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Other diagnostic tests
Differential Diagnosis
Treatment
References
Chapter 58: Palmoplantar Keratodermas
Abstract
Keywords:
Introduction
Hereditary Keratodermas
Diffuse Palmoplantar Keratoderma Without Associated Features (Isolated, Non-Syndromic)
Diffuse Epidermolytic Palmoplantar Keratoderma (EPPK)
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Management
Diffuse Non-epidermolytic Palmoplantar Keratoderma (NEPPK)
Epidemiology and pathogenesis
Clinical features
Differential diagnosis
Pathology
Management
Diffuse Palmoplantar Keratodermas With Ichthyosis
Loricrin Keratoderma
Keratosis Linearis–Ichthyosis Congenita–Sclerosing Keratoderma (KLICK)
Diffuse Palmoplantar Keratodermas With Associated Features/Syndromic
Vohwinkel Syndrome
Mitochondrial Palmoplantar Keratoderma With Hearing Impairment
Huriez Syndrome
Palmoplantar Keratoderma With Sex Reversal and Squamous Cell Carcinoma
Clouston Syndrome
Odonto-onycho-dermal Dysplasia
Olmsted Syndrome
Papillon–Lefèvre and Haim–Munk Syndromes
Naxos Disease
Focal Palmoplantar Keratoderma Without Associated Features (Isolated, Non-Syndromic)
Striate/Focal Palmoplantar Keratoderma
Focal Palmoplantar Keratoderma With Associated Features/Syndromic
Pachyonychia Congenita
History, epidemiology, and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Richner–Hanhart Syndrome
Howel–Evans Syndrome
Carvajal Syndrome
Punctate Palmoplantar Keratoderma Without Associated Features (Isolated, Non-Syndromic)
Punctate Palmoplantar Keratoderma, Buschke–Fischer–Brauer type
Spiny Keratoderma
Marginal Papular Keratoderma: Acrokeratoelastoidosis and Focal Acral Hyperkeratosis
Punctate Palmoplantar Keratoderma With Associated Features/Syndromic
Cole Disease
Acquired Keratodermas and Related Conditions
Keratoderma Climactericum
Keratoderma and Cancer
Other Etiologies of Acquired Keratoderma
Aquagenic Palmoplantar Keratoderma
Circumscribed Palmar or Plantar Hypokeratosis
References
Chapter 59: Darier Disease and Hailey–Hailey Disease
Abstract
Keywords:
Chapter Contents
Darier Disease
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Onset and clinical pattern
Symptoms
Modifying factors
Course of the disease
Infectious complications
Salivary glands
Neuropsychiatric disorders
Others
Clinical subtypes of Darier disease (Table 59.1)
Acral hemorrhagic type
Segmental types 1 and 2
Pathology
Differential Diagnosis
Acrokeratosis verruciformis of Hopf
Others
Treatment
General measures
Topical therapy
Systemic therapy
Surgical therapy
Hailey–Hailey Disease
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Onset and clinical pattern
Symptoms
Modifying factors
Course of the disease
Complications
Infections
Malignant transformation
Clinical subtypes of Hailey–Hailey disease
Segmental type 1
Segmental type 2
Pathology
Differential Diagnosis
Treatment
General measures
Topical and intralesional therapy
Surgical therapy
Systemic therapy
Copyright notice
References
Chapter 60: Primary Immunodeficiencies
Abstract
Keywords
Chapter Contents
Introduction
Ataxia–Telangiectasia
Introduction
Epidemiology
Pathogenesis
Clinical Features
Laboratory Findings
Differential Diagnosis
Treatment
Chronic Mucocutaneous Candidiasis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Laboratory Findings
Differential Diagnosis
Treatment
Cartilage–Hair Hypoplasia Syndrome
Chédiak–Higashi Syndrome
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Complement Disorders
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Chronic Granulomatous Disease
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Hyperimmunoglobulin E Syndromes
Introduction
Epidemiology
Pathogenesis
Clinical Features
Laboratory Findings and Pathology
Differential Diagnosis
Treatment
Immunoglobulin Deficiencies
IPEX Syndrome
Leukocyte Adhesion Deficiency
Introduction
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Severe Combined Immunodeficiency and Omenn Syndrome
Introduction
Epidemiology
Pathogenesis
Clinical Features
Laboratory Findings and Pathology
Differential Diagnosis
Treatment
Wiskott–Aldrich Syndrome
Introduction
Epidemiology
Pathogenesis
Clinical Features
Laboratory Findings and Pathology
Differential Diagnosis
Treatment
References
Chapter 61: Neurofibromatosis and Tuberous Sclerosis Complex
Abstract
Keywords:
Chapter Contents
Introduction
Neurofibromatosis Type 1
History
Epidemiology
Pathogenesis
Clinical Features
Oculocutaneous manifestations
Neurologic manifestations
Skeletal manifestations
Cardiovascular manifestations
Timing of onset of clinical manifestations
Pathology
Differential Diagnosis
Management
Tuberous Sclerosis Complex
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Oculocutaneous manifestations
Neurologic manifestations
Cardiovascular manifestations
Renal manifestations
Other manifestations
Pathology
Differential Diagnosis
Management
References
Chapter 62: Mosaicism and Linear Lesions
Abstract
Keywords:
Chapter Contents
Introduction and History
Patterns and Pathogenesis of Cutaneous Mosaicism
Mosaicism in X-Linked Disorders
Incontinentia Pigmenti
Introduction
History
Genetics
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Hypohidrotic Ectodermal Dysplasia With Immune Deficiency
Goltz Syndrome (Focal Dermal Hypoplasia)
Introduction
Genetics
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Conradi–Hünermann–Happle Syndrome (X-Linked Dominant Chondrodysplasia Punctata)
CHILD Syndrome (Congenital Hemidysplasia With Ichthyosiform Erythroderma [or Nevus] and Limb Defects)
MIDAS Syndrome
Oral–Facial–Digital Syndrome Type I
X-Linked Hypohidrotic Ectodermal Dysplasia (Female Patients)
Menkes Disease (Female Carriers)
X-Linked Reticulate Pigmentary Disorder
Epidermal Nevi and Related Syndromes
Verrucous Epidermal and Sebaceous Nevi
Epidermal Nevus Syndromes
Introduction
History and terminology
Genetics and pathogenesis
Clinical features
Pathology
Management
Proteus Syndrome
Introduction
History
Genetics
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Type 2 Mosaic PTEN Hamartoma Tumor Syndrome
ILVEN (Inflammatory Linear Verrucous Epidermal Nevus)
Mosaicism in Other Autosomal Dominant Disorders
Linear Darier Disease (Acantholytic Dyskeratotic Epidermal Nevus)
Linear Hailey–Hailey Disease (Relapsing Linear Acantholytic Dermatosis)
Linear Porokeratosis
Porokeratotic Adnexal Ostial Nevus (PAON)
Linear Distribution of Basal Cell Carcinomas and Basal Cell Nevi/Basaloid Follicular Hamartomas
Segmental Neurofibromatosis Type 1 and Legius Syndrome
Linear or Segmental Angiofibromas and Mosaicism in Tuberous Sclerosis Complex
Linear or Segmental Benign Tumors
Encephalocraniocutaneous Lipomatosis (Haberland Syndrome)
Vascular Malformations
Venous and Glomuvenous Malformations
Capillary Malformations (Port-Wine Stains)
Pigmented Lesions With a Mosaic Distribution Pattern
Speckled Lentiginous Nevi (SLN), Segmental Agminated Melanocytic Nevi, and Large/Giant Congenital Melanocytic Nevi
McCune–Albright Syndrome
Linear Inflammatory Disorders
Linear Psoriasis
Linear Lichen Planus
Lichen Striatus
“Blaschkitis”
Segmental Vitiligo
Other Linear Inflammatory Disorders
Pigmentary Mosaicism
Linear and Block-Like Hypo- and Hyperpigmentation
Introduction
History
Epidemiology
Genetics
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Nevus Depigmentosus
Chimerism
Block-Like Hyper- and Hypopigmentation Due to Chimerism
References
Chapter 63: Other Genodermatoses
Abstract
Keywords:
Chapter Contents
Introduction
Disorders Featuring Tumorigenesis
Multiple Endocrine Neoplasia
Cowden Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Mucocutaneous manifestations
Extracutaneous manifestations
Pathology
Differential Diagnosis
Treatment
Gardner Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Cutaneous and soft tissue manifestations
Other extraintestinal manifestations
Gastrointestinal manifestations
Pathology
Differential Diagnosis
Treatment
Muir–Torre Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Enzyme Deficiency Diseases
Alkaptonuria
Biotinidase and Holocarboxylase Synthetase Deficiencies
Fabry Disease
Fucosidosis
Gaucher Disease
Hartnup Disease
Mitochondrial Disorders
Niemann–Pick Disease
Phenylketonuria
Premature Aging Syndromes and Poikilodermas
Hutchinson–Gilford Progeria Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology and Laboratory Findings
Differential Diagnosis
Treatment
Werner Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology and Laboratory Findings
Differential Diagnosis
Treatment
Ectodermal Dysplasias
Hypohidrotic Ectodermal Dysplasia
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Hypohidrotic Ectodermal Dysplasia With Immune Deficiency
Pathogenesis
Clinical Features
Treatment
Hidrotic Ectodermal Dysplasia
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Witkop Tooth and Nail Syndrome
Ankyloblepharon–Ectodermal Defects–Cleft Lip/Palate Syndrome
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Ectodermal Dysplasia–Ectrodactyly–Clefting Syndrome
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 64: Developmental Anomalies
Abstract
Keywords:
Chapter Contents
Introduction
The Midline Lesion
Midline Lesions of the Head and Neck
Midline nasal lesions
Midline lesions of the scalp
Other developmental anomalies of the scalp
Dermoid cysts
Cephaloceles
Nasal gliomas
Heterotopic brain tissue and rudimentary meningoceles
Midline cervical clefts
Sternal clefts and supraumbilical raphae
Midline Lesions of the Spine
Aplasia Cutis Congenita
Other Developmental Anomalies
Congenital Lip Pits
Accessory Tragi
Congenital Cartilaginous Rests of the Neck
Branchial Cleft Sinuses and Fistulae
Supernumerary Nipples and Other Forms of Accessory Mammary Tissue
Absent, Hypoplastic or Anomalous Nipples
Skin Dimples
Congenital Malformations of the Dermatoglyphs
Rudimentary Polydactyly
Amniotic Band Sequence and Disorganization Syndrome
Other Developmental Anomalies Associated With Cutaneous Diseases
Section 10: Pigmentary Disorders
Chapter 65: Melanocyte Biology
Abstract
Keywords:
Introduction
Origin and Function of the Melanocyte
Formation and Function of the Melanosome
Regulation of Melanin Biosynthesis
Ultraviolet Radiation (UVR)
Chapter 66: Vitiligo and Other Disorders of Hypopigmentation
Abstract
Keywords
Chapter Contents
Introduction
Diagnosis of Leukodermas
Vitiligo
Epidemiology
Pathogenesis
Genetics of vitiligo
Pathogenic hypotheses for vitiligo
Clinical Features
Clinical variants
Clinical classification of vitiligo
Course of the disease
Vitiligo and ocular disease
Associated disorders
Childhood vitiligo
Pathology
Differential Diagnosis
Treatment
Corticosteroids
Topical calcineurin inhibitors (TCIs)
Photo(chemo)therapy
Narrowband UVB
Psoralen plus UVA
Other phototherapies
Lasers and related light devices
Excimer laser and lamp
Helium–neon laser
Surgical therapies
Combination therapy
Micropigmentation
Depigmentation
Psychological support
Additional controversial therapies
Pseudocatalase with narrowband UVB
Systemic antioxidant therapy
Potential emerging treatments
Ablative laser treatment followed by narrowband UVB plus topical 5-fluorouracil or corticosteroids
Topical prostaglandins
Afamelanotide
Janus kinase (JAK) inhibitors
Hereditary Hypomelanosis
Oculocutaneous Albinism
Epidemiology
Pathogenesis
Clinical features
Ocular manifestations
OCA1
OCA1A
OCA1B
OCA2
OCA3
OCA4
OCA5–7
Ocular albinism type 1 (OA1)
Pathology
Differential diagnosis
Treatment
Piebaldism
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Waardenburg Syndrome
Epidemiology
Pathogenesis
Clinical features
Waardenburg syndrome type 1 (WS1)
Waardenburg syndrome type 2 (WS2)
Klein–Waardenburg syndrome (WS3)
Shah–Waardenburg syndrome (WS4)
Tietz syndrome
Pathology
Diagnosis
Treatment
Disorders of Melanosome Biogenesis
Hermansky–Pudlak syndrome
Pathogenesis
Clinical features
Laboratory findings and pathology
Differential diagnosis
Treatment
Cross syndrome
Chédiak–Higashi syndrome
Tricho-hepato-enteric syndrome (phenotypic diarrhea of infancy)
Disorders of Melanosome Transport and/or Transfer
Epidemiology
Pathogenesis
Clinical features
Treatment
Tuberous Sclerosis Complex
Clinical features
Pathogenesis
Diagnosis
“Pigmentary Mosaicism”: Linear Nevoid Hypopigmentation, Hypomelanosis of Ito, Segmental Pigmentation Disorder, and Nevus Depigmentosus
Clinical features
Pathology
Diagnosis
Nutritional Hypomelanosis
Hypomelanosis Secondary to Cutaneous Inflammation
Epidemiology
Pathogenesis
Clinical features
Pathology
Treatment
Pityriasis Alba
Sarcoidosis
Hypopigmented Mycosis Fungoides
Systemic Sclerosis (Scleroderma) and Lichen Sclerosus
Lupus Erythematosus
Infectious and Parasitic Hypomelanosis
Tinea (Pityriasis) Versicolor
Leprosy (Hansen Disease)
Treponematoses
Onchocerciasis
Post-Kala-Azar Dermal Leishmaniasis
Herpes Zoster
Halo Nevus and Melanoma-Associated Leukoderma
Halo Nevus
Melanoma-Associated Leukoderma
Chemical and Pharmacologic Hypomelanosis
Hypomelanosis From Physical Agents
Miscellaneous
Idiopathic Guttate Hypomelanosis
Vagabond’s Leukomelanoderma
Progressive Macular Hypomelanosis
Hair Hypomelanosis
Leukodermas Without Hypomelanosis
Woronoff’s ring
Nevus anemicus
Cutaneous edema and anemia
Angiospastic macules (Bier spots)
References
Chapter 67: Disorders of Hyperpigmentation
Abstract
Keywords:
Chapter Contents
Diffuse and Circumscribed Hyperpigmentation
Introduction
Postinflammatory Hyperpigmentation
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Erythema Dyschromicum Perstans
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Lichen Planus Pigmentosus
Melasma
Introduction and Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Additional Forms of Circumscribed Hyperpigmentation
Segmental Pigmentation Disorder
Familial Progressive Hyperpigmentation
Primary (Localized) Cutaneous Amyloidosis
Mastocytosis
Tinea (Pityriasis) Versicolor
Atrophoderma of Pasini and Pierini
Pigmented Lesions
Lentigines
Café-au-Lait Macules
Drug-Induced Hyperpigmentation or Discoloration
Additional Forms of Diffuse Hyperpigmentation
Linear Hyperpigmentation
Pigmentary Demarcation Lines
Introduction
Differential Diagnosis
Treatment
Linear Postinflammatory Hyperpigmentation
Clinical Features
Differential Diagnosis
Flagellate Pigmentation From Bleomycin
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Flagellate Mushroom Dermatitis
Introduction
History and Epidemiology
Pathogenesis
Clinical Features
Pathology
Treatment
Hyperpigmentation Along the Lines of Blaschko
Linear and Whorled Nevoid Hypermelanosis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Incontinentia Pigmenti
Reticulated Hyperpigmentation
Prurigo Pigmentosa
Dyskeratosis Congenita
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Naegeli–Franceschetti–Jadassohn Syndrome
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Dermatopathia Pigmentosa Reticularis
Introduction and Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
X-Linked Reticulate Pigmentary Disorder
Introduction and Pathogenesis
Epidemiology
Clinical Features
Pathology
Differential Diagnosis
Treatment
Dowling–Degos Disease
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Reticulate Acropigmentation of Kitamura
Introduction and Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Dyschromatoses
Dyschromatosis Symmetrica Hereditaria
Introduction
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Dyschromatosis Universalis Hereditaria
Introduction and Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Acquired Brachial Cutaneous Dyschromatosis
References
Section 11: Hair, Nails, and Mucous Membranes
Chapter 68: Biology of Hair and Nails
Abstract
Keywords:
Principles of Adnexal Morphogenesis
Biology of Hair
Hair Follicle Morphogenesis
Hair follicle patterning
Postnatal hair follicle development
The Mature Hair Follicle: Cycling and Anatomy
Anagen
Catagen
Telogen
Exogen
Structure of the hair fiber
Hair Keratins
Desmosomes in the Hair Follicle
Hair Follicle Stem Cells
The Hair Follicle and the Immune System
Biology of Nails
Nail Unit Anatomy
Nail Embryology
Nail Matrix
Matrix anatomy
Matrix function
Nail Stem Cells
Nail Unit Melanocytes
The Nail Immune System
Nail Keratins
Other Nail Plate Components
Nail Growth
References
Chapter 69: Alopecias
Abstract
Keywords
Chapter Contents
Introduction
Androgenetic Alopecia
Introduction
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Male pattern hair loss
Female pattern hair loss
Telogen Effluvium
Introduction
Pathogenesis
Clinical Features
Alopecia Areata
Introduction
Epidemiology
Pathogenesis
Clinical Features
Differential Diagnosis
Treatment
Trichotillomania
Introduction
Pathogenesis
Clinical Features
Treatment
Other Non-Cicatricial (Non-Scarring) Alopecias
Pressure-Induced Alopecia
Temporal Triangular Alopecia
Lipedematous Alopecia (Lipedematous Scalp)
Psoriasis of the Scalp and TNF-α Inhibitor-Induced Psoriasiform Alopecia
Congenital Atrichia With Papules
Cicatricial (Scarring) Alopecias
Introduction
Central Centrifugal Cicatricial Alopecia
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lichen Planopilaris and Frontal Fibrosing Alopecia
Introduction
Clinical features
Pathology
Treatment
Discoid Lupus Erythematosus
Introduction
Clinical features
Pathology
Treatment
Acne Keloidalis
Introduction
Pathogenesis
Clinical and histopathologic features
Treatment
Dissecting Cellulitis of the Scalp
Introduction
Clinical and histopathologic features
Differential diagnosis
Treatment
Cicatricial Alopecia, Not Otherwise Classified
“Burnt-out” or “end-stage” cicatricial alopecia
Brocq’s alopecia (pseudopelade of Brocq)
“Folliculitis decalvans”
“Tufted folliculitis”
Traction Alopecia (End-Stage)
Hair Shaft Abnormalities
Introduction
Structural Hair Abnormalities Associated With Increased Hair Fragility
“Bubble hair”
Monilethrix
Pili torti
Trichorrhexis invaginata
Trichorrhexis nodosa
Trichothiodystrophy
Structural Hair Abnormalities Not Associated With Increased Hair Fragility
Acquired progressive kinking of the hair
Loose anagen hair syndrome
Pili annulati
Pili bifurcati
Pili multigemini
Spun-glass hair (uncombable hair)
Woolly hair
Alopecia of Sites Other Than the Scalp
References
Chapter 70: Hypertrichosis and Hirsutism
Abstract
Keywords
Hypertrichosis
Introduction
Clinical Features
Generalized hypertrichosis
Congenital generalized hypertrichosis
Prepubertal hypertrichosis
Acquired generalized hypertrichosis
Acquired hypertrichosis lanuginosa
Localized hypertrichosis
Congenital localized hypertrichosis
Congenital melanocytic nevi and plexiform neurofibromas
Becker melanosis (nevus)
Other hamartomas and infantile tumors
Nevoid hypertrichosis
Spinal dysraphism and the hair collar sign
Localized hypertrichosis in hereditary and acquired systemic diseases
Acquired localized hypertrichosis
Treatment
Hirsutism
Introduction
Epidemiology
Classification
Clinical Features
Constitutional (dermatologic) hirsutism
Adrenal hirsutism
Non-tumoral adrenal hirsutism – adrenal hyperplasias
Hypercortisolism (Cushing syndrome)
Tumoral adrenal hirsutism
Ovarian hirsutism
Non-tumoral ovarian hirsutism
Polycystic ovary syndrome (PCOS)
Ovarian hyperthecosis
Tumoral ovarian hirsutism
Pituitary hirsutism
Iatrogenic hirsutism
Hirsutism due to ectopic hormones
Diagnosis
Treatment
Systemic treatment
Adrenal SAHA syndrome (persistent adrenarche syndrome)
Ovarian SAHA syndrome (excess ovarian androgen release syndrome)
SAHA syndrome with hyperprolactinemia
Iatrogenic hirsutism
Topical medications
Chemical and physical methods
Surgical therapy
References
Chapter 71: Nail Disorders
Abstract
Keywords:
Chapter Contents
Anatomy
Nail Signs
Nail Signs Due to Abnormal Nail Matrix Function
Beau’s lines
Onychomadesis (nail shedding)
Pitting
Onychorrhexis
Trachyonychia (twenty-nail dystrophy, sandpapered nails)
True leukonychia
Koilonychia (spoon nails)
Nail Signs Due to Nail Bed Disorders
Onycholysis
Subungual hyperkeratosis
Apparent leukonychia
Splinter hemorrhages
Nail Signs Due to Deposition of Pigment
Longitudinal melanonychia
Green nail syndrome
Congenital and Hereditary Nail Diseases
Congenital Malalignment of the Great Toenails
Congenital Hypertrophy of the Lateral Fold of the Hallux
Racquet Thumbs (Brachyonychia)
Nail–Patella Syndrome (Onycho-Osteodysplasia; Fong Disease)
Epidermolysis Bullosa
Ectodermal Dysplasias
Pachyonychia Congenita
Darier Disease (Follicular Dyskeratosis)
The Nail in Dermatologic Diseases
Psoriasis
Treatment
Acrodermatitis Continua of Hallopeau (Hallopeau’s Acrodermatitis, Dermatitis Repens)
Treatment
Parakeratosis Pustulosa
Treatment
Lichen Planus
Treatment
Trachyonychia (Twenty-Nail Dystrophy, Sandpapered Nails)
Alopecia Areata
Eczema (Dermatitis)
The Nail in Systemic Diseases
Clubbing (Watch-Glass Nails, Hippocratic Fingers, Drumstick Fingers)
Yellow Nail Syndrome
Treatment
Apparent Leukonychia
Autoimmune Connective Tissue Disorders
HIV Infection
Drug-Induced Nail Abnormalities
Infections
Acute Paronychia
Treatment
Warts
Treatment
Onychomycosis (Tinea Unguium)
Environmental Nail Disorders
Brittle Nails (Fragility, Onychoschizia)
Treatment
Chronic Paronychia
Treatment
Idiopathic Onycholysis
Treatment
Traumatic Nail Abnormalities
Onychotillomania
Treatment
Subungual Hematoma
Treatment
Traumatic Toenail Abnormalities
Onychogryphosis
Pincer Nails (Trumpet Nails)
Treatment
Ingrown Toenails (Onychocryptosis)
Treatment
Nail Tumors
Benign Tumors and Proliferations
Pyogenic granuloma (botryomycoma)
Fibromas/fibrokeratomas
Subungual exostosis
Myxoid cyst (mucous cyst)
Treatment
Glomus tumor
Onychomatricoma
Onychopapilloma
Melanocytic nevi of the nail matrix
Treatment
Malignant Tumors
Bowen disease (squamous cell carcinoma in situ)
Keratoacanthoma
Squamous cell carcinoma
Verrucous carcinoma (carcinoma cuniculatum, epithelioma cuniculatum)
Melanoma
Chapter 72: Oral Disease
Abstract
Keywords:
Chapter Contents
Introduction
Common Benign Conditions of the Oral Cavity
Fordyce Granules
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Geographic Tongue
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Fissured Tongue
Introduction
Epidemiology
Clinical features
Treatment
Hairy Tongue
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Central Papillary Atrophy
Introduction
Clinical features
Pathology
Treatment
Syndromes of the Head and Neck
Basal Cell Nevus Syndrome
Gardner Syndrome
Multiple Endocrine Neoplasia Syndrome Type 2B
Periodontal and Dental Disease
Necrotizing Ulcerative Gingivitis
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Desquamative Gingivitis
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Sequelae of Dental Caries
Introduction
Clinical features
Treatment
Physical and Chemical Injuries
Fibroma
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Chemical Burn
Introduction
Clinical features
Pathology
Treatment
Morsicatio Buccarum
Introduction
Clinical features
Pathology
Treatment
Traumatic Ulcer
Introduction
Clinical features
Treatment
Drug-Related Gingival Enlargement (Hyperplasia)
Introduction
Clinical features
Pathology
Treatment
Allergic and Immunologic Diseases
Contact Stomatitis
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Recurrent Aphthous Stomatitis
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Behçet Disease
Eosinophilic Ulcer of the Oral Mucosa
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Orofacial Granulomatosis
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Granulomatosis With Polyangiitis (Formerly Wegener Granulomatosis)
Introduction/clinical features
Pathology
Treatment
Epithelial Pathology
Oral Leukoplakia/Erythroplakia
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Proliferative Verrucous Leukoplakia
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Nicotine Stomatitis
Introduction
Clinical features
Pathology
Treatment
Actinic Cheilitis
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Oral Squamous Cell Carcinoma
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Verrucous Carcinoma
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Salivary Gland Diseases
Mucocele
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Necrotizing Sialometaplasia
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Sjögren Syndrome
Introduction/clinical features
Pathology
Treatment
Salivary Gland Tumors
Introduction/epidemiology
Clinical features/pathology
Treatment
Oral Manifestations of Hematologic/Oncologic Disease
Chemotherapy- and Radiotherapy-Induced Oral Mucositis
Introduction
Clinical features
Treatment
Leukemia
Introduction
Clinical features
Treatment
Lymphoma
Introduction/epidemiology
Clinical features
Pathology
Treatment
Melanoma
Introduction/epidemiology
Clinical features
Pathology
Treatment
Oral Manifestations of Systemic Disease
Amyloidosis
Introduction
Clinical features
Pernicious Anemia
Introduction
Epidemiology
Clinical features
Treatment
Crohn Disease
Introduction/epidemiology
Clinical features
Pathology
Treatment
Pyostomatitis Vegetans
Introduction
Epidemiology
Clinical features
Pathology
Treatment
Oral Manifestions of HIV Infection
Chapter 73: Anogenital (Non-venereal) Disease
Abstract
Keywords:
Chapter Contents
Introduction
Lichen Sclerosus
Introduction
History
Epidemiology
Etiology
Clinical features
Pathology
Differential diagnosis
Treatment
Lichen Planus
Introduction
History
Epidemiology
Etiology
Clinical features
Pathology
Differential diagnosis
Treatment
Zoon Balanitis/Vulvitis
Introduction
Epidemiology
Etiology
Clinical features
Pathology
Differential diagnosis
Treatment
Epithelial Disorders of the Anogenital Region
Dermatitis
Introduction
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Contact Dermatitis
Psoriasis
Clinical features
Differential diagnosis
Treatment
Premalignant and Malignant Lesions
Intraepithelial Neoplasia (Vulvar/Vulval, Penile, Anal)
Introduction
Epidemiology
Pathogenesis
Pathology
Clinical features
Differential diagnosis
Treatment
Prevention
Pseudoepitheliomatous Keratotic and Micaceous Balanitis
Invasive Squamous Cell Carcinomas
Epidemiology
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Anogenital Melanoma
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Basal Cell Carcinoma
Extramammary Paget Disease
Introduction
Epidemiology
Etiology
Clinical features
Pathology
Differential diagnosis
Treatment
Dysesthetic Genital Pain Syndromes
Localized Vulvodynia (Vestibulodynia)
Introduction
Clinical features
Differential diagnosis
Treatment
Generalized Vulvodynia/Scrotodynia (Dysesthetic Vulvodynia/Scrotodynia)
Introduction
Clinical features
Differential diagnosis
Treatment
Cyclical Vulvovaginitis
Anal Pain
Benign Lesions
Epidermoid Cysts
Vestibular Papillomatosis
The Anogenital Region and Systemic Disease
Infections
Perianal Streptococcal Disease
Recurrent Toxin-Mediated Perineal Erythema
Kawasaki Disease
Fournier Gangrene
Erythrasma
Dermatophytosis
Cutaneous Candidiasis
Introduction
Epidemiology
Etiology
Clinical features
Differential diagnosis
Treatment
Recurrent Candidiasis
Erosive Genital Disease
Acquired Autoimmune Bullous Diseases
Acquired Inflammatory Bullous Diseases
Erythema multiforme and Stevens–Johnson syndrome
Fixed drug eruption
Inherited Bullous Disease
Hailey–Hailey disease
References
Section 12: Infections, Infestations, and Bites
Chapter 74: Bacterial Diseases
Abstract
Keywords
Chapter Contents
The Cutaneous Microbiota
Gram-Positive Bacteria
Staphylococcal and Streptococcal Skin Infections
Impetigo
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Bacterial Folliculitis
Introduction
Epidemiology and pathogenesis
Clinical features
Diagnosis and differential diagnosis
Treatment
Abscesses, Furuncles, and Carbuncles
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Methicillin-Resistant Staphylococcus aureus
Introduction
Clinical features
Pathogenesis
Diagnosis and treatment
Blistering Distal Dactylitis
Ecthyma
Staphylococcal Scalded Skin Syndrome
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Toxic Shock Syndrome
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Scarlet Fever
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Streptococcal Toxic Shock Syndrome
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Erysipelas
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Streptococcal Intertrigo
Perianal and Vulvovaginal (Perineal) Streptococcal Infection
Cellulitis
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Pyomyositis
Botryomycosis
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Necrotizing Fasciitis
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Bacteremia/Septicemia
Clostridial Skin Infections
Corynebacterial Skin Infections
Erythrasma
Pitted Keratolysis
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Trichomycosis Axillaris, Pubis, and Capitis
Cutaneous Diphtheria
Other Gram-Positive Skin Infections
Cutaneous Anthrax
Introduction and epidemiology
History
Pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Bacillus cereus Infection
Erysipeloid
Listeriosis
Gram-Negative Bacteria
Neisseria Meningitidis
Acute and Chronic Meningococcemia
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Pseudomonas Aeruginosa
Green Nail Syndrome
Pseudomonal Pyoderma and Blastomycosis-like Pyoderma
Otitis Externa and Malignant Otitis Externa
Pseudomonal Folliculitis
Pseudomonas Hot-Foot Syndrome
Ecthyma Gangrenosum
Bartonella
Bartonellosis
Introduction
History
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Cat Scratch Disease
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Bacillary Angiomatosis
Introduction
History
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Other Gram-Negative Skin Infections
Brucellosis
Glanders
Melioidosis
Cutaneous Malacoplakia (Malakoplakia)
Tularemia
Haemophilus influenzae Cellulitis
Rhinoscleroma
Salmonellosis
Rat-Bite Fever
Plague
Vibrio vulnificus Infection
Ehrlichiosis and Anaplasmosis
Spirochetes
Borrelia Burgdorferi
Lyme Disease
Borrelial Lymphocytoma
Acrodermatitis Chronica Atrophicans
Non-Venereal (Endemic) Treponematoses
Yaws
Pinta
Endemic Syphilis
Leptospirosis
Bacteria Previously Classified as Fungi
Actinomycosis
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Nocardiosis
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
References
Chapter 75: Mycobacterial Infections
Abstract
Keywords:
Chapter Contents
Leprosy
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology and Laboratory Evaluation
Differential Diagnosis
Treatment
Cutaneous Tuberculosis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Cutaneous tuberculosis
Tuberculids
BCG inoculation
Diagnosis
Treatment
Non-Tuberculous Mycobacterioses
Introduction
Mycobacterium ulcerans
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Mycobacterium marinum
Introduction and history
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Mycobacterium kansasii
Introduction and history
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Mycobacterium fortuitum, Mycobacterium chelonae, and Mycobacterium abscessus
Introduction and history
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Mycobacterium avium Complex (MAC)
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Mycobacterium haemophilum
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Mycobacterium scrofulaceum
Introduction
Epidemiology and pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Chapter 76: Rickettsial Diseases
Abstract
Keywords:
Chapter Contents
Introduction
Spotted Fever and Typhus Group Rickettsial Infections
History
Epidemiology
Pathogenesis
Clinical Features
Clinical course and systemic features
Shared cutaneous features
Features of specific diseases
Pathology
Diagnosis and Differential Diagnosis
Treatment
Scrub Typhus
Human Ehrlichioses
Human Granulocytic Anaplasmosis
Q Fever
References
Chapter 77: Fungal Diseases
Abstract
Keywords:
Superficial Mycoses
Introduction
Non-inflammatory superficial mycoses
History
Epidemiology
Pathogenesis
Clinical features
Piedra
Tinea nigra
Tinea (pityriasis) versicolor
Malassezia (Pityrosporum) folliculitis
Pathology and fungal culture
Differential diagnosis
Treatment
Dermatophytoses
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Tinea corporis
Tinea cruris
Tinea manuum
Tinea barbae
Tinea faciei
Tinea capitis
Tinea pedis
Tinea unguium (dermatophytic onychomycosis)
Pathology
Differential diagnosis
Treatment
Invasive dermatophytosis
Mucocutaneous candida infections
Chronic mucocutaneous candidiasis
Granuloma gluteale infantum/adultorum and perianal pseudoverrucous papules/nodules
Subcutaneous Mycoses
Chromoblastomycosis
Introduction
History
Epidemiology and pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Mycetoma
Introduction
History and epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Sporotrichosis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lobomycosis
Systemic Mycoses
True Pathogens
Introduction
Histoplasmosis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Blastomycosis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Coccidioidomycosis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Paracoccidioidomycosis
Introduction and epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Opportunistic Pathogens
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Phaeohyphomycosis
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Pneumocystis
References
Chapter 78: Cutaneous Manifestations of HIV Infection
Abstract
Keywords
Introduction
History
Epidemiology
Pathogenesis
HIV-Related Infectious Skin Conditions
Viral Infections
Primary HIV exanthem (acute retroviral syndrome)
Herpes simplex virus (HSV)
Varicella–zoster virus (VZV)
Molluscum contagiosum virus
Human papillomavirus (HPV)
Epstein–Barr virus (EBV)
Cytomegalovirus (CMV)
Bacterial Infections
Staphylococcus aureus
Bacillary angiomatosis
Mycobacteria
Syphilis
Fungal and Related Infections
Candidiasis
Dermatophytoses
Systemic fungal infections
Pneumocystis jiroveci
Other fungal and related infections
Parasitic Infections
Leishmaniasis
Strongyloidiasis
Acanthamebiasis
Ectoparasitic Infestations and Insect Bites
Scabies
Demodicosis
Insect bite reactions
Non-Infectious HIV-Related Dermatoses
Papulosquamous Disorders
Seborrheic dermatitis
Psoriasis
Reactive arthritis
Other papulosquamous dermatoses
Non-Infectious Pruritic Papular Disorders
Pruritic papular eruption
Eosinophilic folliculitis
Hair and Nail Disorders
Vasculitis
Photosensitivity Reactions
UV light hypersensitivity
Porphyria cutanea tarda (PCT)
Chronic actinic dermatitis
Metabolic Changes
HIV/ART-associated lipodystrophy
Malnutrition
Miscellaneous Dermatoses
HIV-Related Cutaneous Neoplasms
Kaposi Sarcoma (KS)
Squamous and Basal Cell Carcinomas and Melanoma
Lymphomas
Other Cutaneous Neoplasms
Differential Diagnosis and Diagnosis
Differential Diagnosis of HIV-associated Skin Conditions
Diagnosis of HIV-associated Skin Conditions
Laboratory Diagnosis of HIV Infection
Antiretroviral Therapy (ART)
Antiretroviral Medications for Treatment and Prevention
Immune Reconstitution Inflammatory Syndrome (IRIS)
Drug–Drug Interactions
Drug Eruptions
Conclusions
References
Chapter 79: Human Papillomaviruses
Abstract
Keywords
Introduction
History
Epidemiology
Pathogenesis
Papillomavirus Evolution
Virology
Papillomavirus Life Cycle
Host Immune Response
Oncogenic Potential of HPV
Clinical Features
Cutaneous Infections
Mucosal Infections
Pathology
Common and Deep Palmoplantar Warts
Flat Warts
Epidermodysplasia Verruciformis
Anogenital Warts
Giant Condylomata of Buschke–Löwenstein
Squamous Intraepithelial Neoplasias
Differential Diagnosis
Common Warts
Anogenital Lesions
Oral Warts
Therapy
General Considerations
Local Destructive Therapy
Topical and Intralesional Cytotoxic Therapy
Topical Immunomodifiers
Systemic Immunomodifiers
Immunotherapy
Antiviral Agents
Retinoids
Special Considerations
HPV Vaccines
Disclaimer
References
Chapter 80: Human Herpesviruses
Abstract
Keywords:
Chapter Contents
Herpes Simplex Viruses (HSV-1 and HSV-2)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Other Clinical Presentations of HSV Infection
Diagnosis and Pathology
Differential Diagnosis
Treatment and Prevention
Varicella–Zoster Virus (VZV; HHV-3)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Varicella
Herpes zoster
Diagnosis and Pathology
Differential Diagnosis
Treatment and Prevention
Varicella
Herpes zoster
Epstein–Barr Virus (HHV-4)
Introduction and History
Epidemiology
Pathogenesis
Clinical Features
Diagnosis and Pathology
Differential Diagnosis
Treatment
Cytomegalovirus (HHV-5)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Diagnosis and Pathology
Differential Diagnosis
Treatment and Prevention
Human Herpesvirus Type 6 (HHV-6)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Diagnosis and Pathology
Differential Diagnosis
Treatment
Human Herpesvirus Type 7 (HHV-7)
Human Herpesvirus Type 8 (HHV-8)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Diagnosis and Pathology
Treatment
Conclusion
References
Chapter 81: Other Viral Diseases
Abstract
Keywords:
Chapter Contents
Introduction
Enterovirus Infections
Introduction
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
Measles
Introduction and History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
Rubella
Introduction and History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
Erythema Infectiosum
Introduction and History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
Roseola Infantum
Unilateral Laterothoracic Exanthem
Introduction and History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
Gianotti–Crosti Syndrome
Introduction and History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
Poxvirus Infections
Viral Hemorrhagic Fevers, Including Dengue
West Nile Virus Infection
Zika Virus Infection
Chikungunya Fever
Barmah Forest and Ross River Virus Infections
Cutaneous Manifestations of Hepatitis A, B, and C Infection
Trichodysplasia Spinulosa
Rabies
Kawasaki Disease
Introduction and History
Epidemiology
Pathogenesis
Clinical Features and Differential Diagnosis
Pathology
Treatment
References
Chapter 82: Sexually Transmitted Infections
Abstract
Keywords
Chapter Contents
Introduction
Syphilis
History
Epidemiology
Biology of T. pallidum
Pathogenesis of Untreated Syphilis
Primary stage
Secondary stage
Latency
Tertiary stage
Syphilis and HIV
Clinical Features
Primary syphilis
Secondary syphilis
Latent syphilis
Tertiary (late) syphilis
“Benign” late syphilis
Cardiovascular syphilis
Neurosyphilis
Neurosyphilis in HIV-infected individuals
Laboratory diagnosis of neurosyphilis
Congenital syphilis
Early congenital syphilis
Late congenital syphilis and stigmata
Laboratory diagnosis of congenital syphilis
Laboratory Diagnosis of Syphilis
Identification of T. pallidum
Microscopic examination
Polymerase chain reaction-based assays
Serologic tests for syphilis
Non-treponemal tests
Treponemal tests
Pathology
Differential Diagnosis
Treatment
Gonorrhea
History
Epidemiology
Transmission
Biology of N. gonorrhoeae
Pathogenesis
Clinical Features
Gonococcal infection in men
Gonococcal infection in women
Extragenital gonorrhea
Pharyngeal gonorrhea
Rectal gonorrhea
Gonococcal ophthalmia (including ophthalmia neonatorum)
Disseminated gonococcal infection
Arthritis–dermatosis syndrome (gonococcemia)
Laboratory Diagnosis
Staining methods
Non-culture techniques for gonococcal diagnosis
Nucleic acid amplification techniques (NAATs)
Non-amplified DNA hybridization
Culture techniques
Pathology
Differential Diagnosis
Treatment
Chancroid
History
Epidemiology
Transmission
Chancroid and HIV
Biology of the Organism
Pathogenesis
Clinical Features
Pathology
Diagnosis
Differential Diagnosis
Treatment
Lymphogranuloma Venereum
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Diagnosis
Differential Diagnosis
Treatment
Donovanosis (Granuloma Inguinale)
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Diagnosis
Differential Diagnosis
Treatment
References
Chapter 83: Protozoa and Worms
Abstract
Keywords
Chapter Contents
Protozoa
Leishmaniasis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Cutaneous leishmaniasis
Mucocutaneous/mucosal leishmaniasis
Visceral leishmaniasis (kala-azar)
Leishmaniasis and HIV
Pathology
Diagnosis
Differential Diagnosis
Treatment
Cutaneous Diseases Caused by Amebas
Amebiasis due to Entamoeba Histolytica
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology and diagnosis
Differential diagnosis
Treatment
Balamuthia mandrillaris Infection
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology and diagnosis
Treatment
Trypanosomiasis
Chagas Disease
Epidemiology
Pathogenesis
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
African Trypanosomiasis
Epidemiology
Pathogenesis
Clinical features
Pathology and diagnosis
Differential diagnosis
Treatment
Toxoplasmosis
Worms (Helminths)
Cutaneous Larva Migrans
Epidemiology and Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Gnathostomiasis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Diagnosis and Differential Diagnosis
Treatment
Onchocerciasis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Diagnosis
Differential Diagnosis
Treatment
Filariasis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Diagnosis
Differential Diagnosis
Treatment
Schistosomiasis and Swimmer’s Itch
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Diagnosis
Differential Diagnosis
Treatment
Cysticercosis and Echinococcosis
Introduction
Epidemiology
Pathogenesis
Clinical Features
Diagnosis
Treatment
References
Chapter 84: Infestations
Abstract
Keywords:
Chapter Contents
Scabies
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Permethrin
Lindane
Crotamiton
Sulfur ointment
Ivermectin
Head Lice
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pyrethrins
Permethrin
Lindane
Malathion
Carbaryl
Ivermectin
Benzyl alcohol
Dimethicone
Spinosad
Crab Lice
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Body Lice
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Tungiasis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Cutaneous Myiasis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Preventative Measures
References
Chapter 85: Bites and Stings
Abstract
Keywords:
Chapter Contents
Insects
Insect Bites and Stings (Class Insecta)
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Fire Ants
Introduction
Pathogenesis
Clinical features
Treatment
Bedbugs
Introduction
Pathogenesis
Clinical features
Treatment
Triatomine (Reduviid) Bug
Introduction
Clinical features
Blister Beetles
Introduction
Clinical features
Pathology
Treatment
Rove Beetles
Introduction
Clinical features
Pathology
Treatment
Fleas
Introduction
Clinical features
Treatment
Lepidopterism
Introduction
Pathogenesis
Clinical features
Arachnids
Tick Bites (Class Arachnida: Subclass Acarina)
Introduction
Clinical features
Treatment
Environmental measures
Repellents
Tick removal
Antibiotic prophylaxis after tick bites
Amblyomma Ticks
Introduction
Clinical features
Dermacentor Ticks
Introduction
Pathogenesis
Clinical features
Rhipicephalus Ticks
Introduction
Clinical features
Ixodes Ticks
Introduction
Clinical features
Mites (Class Arachnida: Subclass Acarina)
Introduction
Clinical features
Differential diagnosis
Treatment
Chiggers
Introduction
Clinical features
Treatment
Cheyletiella
Introduction
Clinical features
Treatment
Spider Bites (Class Arachnida: Order Araneae)
Widow Spiders (Latrodectus)
Introduction
Pathogenesis
Clinical features
Treatment
Loxosceles Spiders
Introduction
Pathogenesis
Clinical features
Pathology
Treatment
Funnel Web Spiders
Introduction
Pathogenesis
Clinical features
Tarantulas (Family Theraphosidae)
Introduction
Clinical features
Pathology
Scorpions (Class Arachnida: Order Scorpiones)
Introduction
Clinical features
Treatment
Centipedes (Class Chilopoda) and Millipedes (Class Diplopoda)
Introduction
Clinical features
Treatment
Snake Bites
Introduction
Clinical features
Treatment
Dog and Cat Bites
Introduction
Clinical features
Treatment
Leeches
Introduction
Pathogenesis
Clinical features
Treatment
Leech therapy
Marine Injuries
Introduction
Pathogenesis
Clinical features
Pathology
Treatment
References
Section 13: Disorders Due to Physical Agents
Chapter 86: Ultraviolet Radiation
Abstract
Keywords:
Introduction
Spectrum of UV Light and Absorption Spectra
Short-Term Effects of Ultraviolet Light: Sunburn and Tanning
Effects on the Cutaneous Immune System
Long-Term Effects of Ultraviolet Light: Photoaging and Photocarcinogenesis
Photoaging
UV-Induced Tumor Formation
UV Induction of DNA Damage
Repair of UV-Induced DNA Damage
UV-Induced Mutation Formation
Protection Against Photocarcinogenesis
References
Chapter 87: Photodermatologic Disorders
Abstract
Keywords:
Abnormal Cutaneous Effects to Light
Immunologically-Mediated Photodermatoses
Polymorphous light eruption
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Actinic prurigo
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Hydroa vacciniforme
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Chronic actinic dermatitis
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Solar urticaria
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Photosensitity Due to Hereditary Defects in DNA Repair
Xeroderma pigmentosum
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Treatment
Cockayne syndrome
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Treatment
UV-sensitive syndrome
Trichothiodystrophy
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Treatment
Photosensitivity Due to Chromosomal Instability
Bloom syndrome
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Treatment
Rothmund–Thomson syndrome
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Chemical- and Drug-Induced Photosensitivity
Exogenous photosensitivity
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Phototoxicity
Photoallergy
Pathology
Differential diagnosis
Treatment
Endogenous photosensitivity
Photoaggravated Dermatoses
Introduction and history
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Grover Disease
Normal Cutaneous Effects of UVR Exposure
Acute and Subacute Effects
Acute inflammatory erythema (sunburn)
Pigment darkening and delayed tanning
Epidermal hyperplasia
Immunologic changes
Vitamin D3 synthesis
Other changes
Chronic Effects
Photoaging in fair-skinned patients
Photoaging in patients with darker skin phototypes
Photocarcinogenesis
References
Chapter 88: Environmental and Sports-Related Skin Diseases
Abstract
Keywords:
Chapter Contents
Injury Due to Heat Exposure
Heat-Related Illnesses and Thermal Burns
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Erythema Ab Igne
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Burns Associated With Fluoroscopy and Magnetic Resonance Imaging (MRI)
Introduction
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Airbag Burns
Introduction
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Injury Due to Cold Exposure
Frostbite
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Pernio
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Injury Due to Water Exposure
Immersion Foot
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Injury Due to Electricity
Electrical Burns
Introduction
Pathogenesis
Clinical features
Pathology
Treatment
Chemical Burns and Exposures
Injury Due to Chemical Exposure
Chemical Hair Discoloration
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Arsenical and Heavy Metal Dermatoses
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Illness due to Aquatic Toxin Ingestion
Introduction
Epidemiology
Pathogenesis
Clinical features
Treatment
Frictional and Traumatic Injury to the Skin
Corns and Calluses
Introduction
Epidemiology
Pathogenesis
Clinical features
Differential diagnosis
Treatment
Black Heel and Palm
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Senile Gluteal Dermatosis
Environmental Dermatoses of the External Ear
Sports-Related Dermatoses
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Treatment
Amputee-Related Dermatoses
Treatment
Musical Instrument-Related Dermatoses
Introduction
History
Epidemiology
Pathogenesis and clinical features
Pathology
Differential diagnosis
Treatment
Appendix
References
Chapter 89: Signs of Drug Abuse
Abstract
Keywords:
Introduction
Amphetamines
Benzodiazepines
Cannabis
Cocaine
Gamma-Hydroxybutyrate
Lysergic Acid Diethylamide
Opioids (Includes Opiates)
Anabolic Steroids
Pathophysiology of Addiction
Epidemiology
Clinical Features
Scars
Bacterial Infections
Fungal Infections
Granulomas
Mucous Membrane Lesions
Burns
Ulcers
Formication
Additional Cutaneous Findings
Drug-Induced Reactions
Vascular Lesions
Systemic Complications
Treatment
References
Chapter 90: Skin Signs of Abuse
Abstract
Keywords
Chapter Contents
Child Abuse
Introduction
History
Epidemiology
Clinical Features
Physical abuse
Physical neglect
Emotional deprivation
Sexual abuse
Differential Diagnosis
Evaluation and Management
Elder Abuse
Introduction
Risk Factors for Elder Abuse
Clinical Features and Assessment
Management
References
Section 14: Disorders of Langerhans Cells and Macrophages
Chapter 91: Histiocytoses
Abstract
Keywords:
Overview
Langerhans Cell Histiocytosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Non-Langerhans Cell Histiocytoses
Benign Cephalic Histiocytosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Generalized Eruptive Histiocytoma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Indeterminate Cell Histiocytosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Juvenile Xanthogranuloma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Necrobiotic Xanthogranuloma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Reticulohistiocytosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Rosai–Dorfman Disease
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Xanthoma Disseminatum
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Malignant Histiocytic Disorders
Dermal Dendrocyte Hamartomas
References
Chapter 92: Xanthomas
Abstract
Keywords:
Introduction
Epidemiology
Pathogenesis
Clinical Features
Eruptive Xanthomas
Tuberous/Tuberoeruptive Xanthomas
Tendinous Xanthomas
Plane Xanthomas and Xanthelasma
Verruciform Xanthomas
Pathology
Differential Diagnosis
Treatment
Acknowledgment
References
Chapter 93: Non-infectious Granulomas
Abstract
Keywords:
Chapter Contents
Introduction
Sarcoidosis
History
Epidemiology
Pathogenesis
Immune mechanisms
Triggers
Genetics
Clinical Features
Pathology
Diagnosis and Differential Diagnosis
Treatment
Granuloma Annulare
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Annular Elastolytic Giant Cell Granuloma
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Necrobiosis Lipoidica
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Cutaneous Crohn Disease
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Foreign Body Granulomas (see Ch. 94)
Interstitial Granulomatous Dermatitis and Palisaded Neutrophilic and Granulomatous Dermatitis
Introduction
History
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 94: Foreign Body Reactions
Abstract
Keywords:
Chapter Contents
General Considerations
Definition
Routes of Entry
Accidental
Surgical procedures
Iatrogenic
Tattooing
Topical application
Self-inflicted
Pathogenesis
Clinical Features
Pathology
Diagnosis
Foreign Body Reactions to Inorganic and Metallic Compounds
Tattoo
Clinical features
Pathology
Diagnosis and differential diagnosis
Treatment
Paraffin
Clinical features
Pathology
Treatment
Silicone
Clinical features
Pathology
Treatment
Silica
Clinical features
Pathology
Differential diagnosis
Treatment
Talc
Clinical features
Pathology
Treatment
Zirconium
Beryllium
Aluminum
Zinc
Reactions to Organic and Biologic Products
Starch
Pathology
Cactus Spines
Pathology
Cnidaria (Coelenterata) – Jellyfish, Corals
Clinical features
Pathology
Treatment
Keratin
Pseudofolliculitis barbae and acne keloidalis nuchae
Treatment
Pilonidal sinus
Injectable Soft Tissue Fillers
Hyaluronic Acid
Bovine Collagen
Miscellaneous
Intralesional Corticosteroids
Sutures
Polyamide “Hair” Implants
Mesotherapy
References
Section 15: Atrophies and Disorders of Dermal Connective Tissues
Chapter 95: Biology of the Extracellular Matrix
Abstract
Keywords
Introduction
Structure and Function of the Extracellular Matrix
Collagens
Collagen triple helix
Biosynthesis of collagens
The collagen family of proteins
Collagens of the skin
Elastic Fibers
Elastin
Microfibrils
The Extrafibrillar Matrix
Glycosaminoglycans and proteoglycans
Laminins and Other Glycoproteins
Functions of the Extracellular Matrix
Structural role of the extracellular matrix
Regulation of cellular functions
Diseases Related to ECM Defects
Ehlers–Danlos syndrome
Collagens and tenascin-X
Post-translational modification of collagen
Proteoglycan processing
Cutis laxa
Marfan syndrome
Loeys–Dietz syndrome
Other fibrillin-associated syndromes
Pseudoxanthoma elasticum
Epidermolysis bullosa
Other hereditary matrix diseases with skin symptoms
Extracellular matrix components as autoantigens
Skin aging and other acquired ECM abnormalities
Animal Models
Potential for Biologically Valid Molecular Therapies
References
Chapter 96: Perforating Diseases
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Reactive Perforating Collagenosis
Elastosis Perforans Serpiginosa
Acquired Perforating Dermatosis and Kyrle Disease
Perforating Calcific Elastosis
Pathology
Differential Diagnosis
Treatment
References
Chapter 97: Heritable Disorders of Connective Tissue
Abstract
Keywords
Chapter Contents
Introduction
Ehlers–Danlos Syndrome
History
Epidemiology
Pathogenesis
Clinical Features
Pathology and Laboratory Findings
Differential Diagnosis
Treatment
Pseudoxanthoma Elasticum
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Cutis Laxa
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 98: Dermal Hypertrophies
Abstract
Keywords:
Chapter Contents
Hypertrophic Scars and Keloids
Introduction
Epidemiology
Etiology
Pathogenesis
Clinical Features
Scar assessment
Pathology
Differential Diagnosis
Treatment
Dupuytren Disease
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Cutis Verticis Gyrata
Clinical Features
Pathology
Differential Diagnosis
Treatment
Hyaline Fibromatosis Syndrome (Juvenile Hyaline Fibromatosis and Infantile Systemic Hyalinosis)
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Elastic Tissue-Related Disorders
References
Chapter 99: Atrophies of Connective Tissue
Abstract
Keywords:
Chapter Contents
Mid-Dermal Elastolysis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Anetoderma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Striae
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Atrophoderma of Pasini and Pierini
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Follicular Atrophoderma
Clinical features
Atrophoderma Vermiculatum
Bazex–Dupré–Christol Syndrome
Conradi–Hünermann–Happle Syndrome (X-Linked Dominant Chondrodysplasia Punctata, CDPX2)
Atrophia Maculosa Varioliformis Cutis
Piezogenic Pedal Papules (Piezogenic Papules)
Other Atrophies of the Connective Tissue
References
Section 16: Disorders of Subcutaneous Fat
Chapter 100: Panniculitis
Abstract
Keywords:
Chapter Contents
Introduction
Erythema Nodosum
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Subacute Nodular Migratory Panniculitis
Clinical features
Pathology
Treatment
Morphea/Scleroderma Panniculitis
Clinical features
Pathology
Differential diagnosis
Alpha-1 Antitrypsin Deficiency Panniculitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Erythema Induratum
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Pancreatic Panniculitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Sclerema Neonatorum, Subcutaneous Fat Necrosis of the Newborn, and Post-Steroid Panniculitis
Sclerema Neonatorum
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Subcutaneous Fat Necrosis of the Newborn
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Post-Steroid Panniculitis
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lupus Erythematosus Panniculitis (Lupus Panniculitis)
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Panniculitis of Dermatomyositis
Introduction
Clinical features
Pathology
Differential diagnosis
Treatment
Traumatic Panniculitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lipodermatosclerosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Infection-Induced Panniculitis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Cytophagic Histiocytic Panniculitis
Malignant Subcutaneous Infiltrates
Unusual or Newly Described Forms of Panniculitis
References
Chapter 101: Lipodystrophies
Abstract
Keywords:
Introduction
Pathogenesis
Congenital Generalized Lipodystrophy (Berardinelli–Seip Congenital Lipodystrophy, Berardinelli–Seip Syndrome)
Familial Partial Lipodystrophy
Acquired Generalized Lipodystrophy (Lawrence Syndrome)
Acquired Partial Lipodystrophy Syndrome (Barraquer–Simons Syndrome)
Localized Lipoatrophy
Clinical Features
Congenital Generalized Lipodystrophy (CGL)
Familial Partial Lipodystrophy
Autoinflammatory Syndromes (see Table 45.7)
Joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced lipodystrophy (JMP) syndrome
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome
Recurrent lipoatrophic panniculitis of children
Acquired Generalized Lipodystrophy
Acquired Partial Lipodystrophy
Localized Lipoatrophy
Lipohypertrophy
Pathology
Differential Diagnosis
Treatment
Acquired Partial Lipodystrophy
Insulin Lipodystrophy
HIV/ART-Associated Lipodystrophy
Pathogenesis
Clinical Features
Treatment
HIV/ART-associated lipoatrophy
HIV/ART-associated lipohypertrophy
Metabolic derangements
References
Section 17: Vascular Disorders
Chapter 102: Vascular Biology
Abstract
Keywords:
Chapter Contents
Structure and Function of Cutaneous Blood Vessels
Vascular Development
Regulation of Skin Angiogenesis
Regulation of Leukocyte–Endothelial Cell Interactions
Structure and Function of the Cutaneous Lymphatic Vascular System
Related Diseases
Current Trends in Antiangiogenesis
References
Chapter 103: Infantile Hemangiomas
Abstract
Keywords
Introduction
History
Epidemiology
Pathogenesis
Hemangioma Endothelial Cells: Origin, Signaling Pathways, and Genetics
Hypoxia and Other Extrinsic Factors
Factors Influencing Natural History
Clinical Features
Presentation
Natural History
Complications
Ulceration
Disfigurement and interference with function
Disfigurement and interference with function because of location
Extracutaneous involvement
Radiologic Features
Pathology
Differential Diagnosis
Treatment
Active Non-Intervention
Management of Ulceration
Local Medical Therapies
Topical β-blockers
Intralesional and topical corticosteroids
Systemic Therapies
Systemic β-blockers
Systemic corticosteroids
Other systemic therapies
Surgical and Laser Therapy
Rapidly Involuting and Non-Involuting Congenital Hemangiomas
References
Chapter 104: Vascular Malformations
Abstract
Keywords
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Capillary Malformations
Nevus simplex
Port-wine stain and variants
Sturge–Weber syndrome
Capillary malformations associated with overgrowth
Diffuse capillary malformation with overgrowth
Klippel–Trenaunay syndrome
Proteus syndrome
CLOVES syndrome
Megalencephaly–capillary malformation
CLAPO syndrome
Cutis marmorata telangiectatica congenita
Telangiectasias
Hereditary hemorrhagic telangiectasia
Ataxia–telangiectasia
Angiokeratomas
Venous Malformations
Cephalic venous malformation
Trunk and limb venous malformation
Sinusoidal hemangioma
Fibro-adipose vascular anomaly
Syndromes associated with venous malformations
Familial cutaneous and mucosal venous malformation (VMCM)
Blue rubber bleb nevus syndrome (BRBNS; Bean syndrome)
Maffucci syndrome
Glomuvenous malformation
Cerebral cavernous malformation and hyperkeratotic cutaneous capillary–venous malformation
Lymphatic Malformations
Primary lymphedema
Macrocystic lymphatic malformation (“cystic hygroma”)
Microcystic lymphatic malformation (“lymphangioma circumscriptum”)
Combined microcystic and macrocystic lymphatic malformation
Generalized lymphatic anomaly
Gorham–Stout disease
Kaposiform lymphangiomatosis
Arteriovenous Malformations
Syndromes associated with arteriovenous malformations
Cobb syndrome
Bonnet–Dechaume–Blanc syndrome (BDBS; Wyburn–Mason syndrome)
Parkes Weber syndrome
Capillary malformation–arteriovenous malformation (CM-AVM)
PTEN hamartoma tumor syndrome (PHTS)
Pathology
Capillary Malformations
Venous Malformations
Lymphatic Malformations
Arteriovenous Malformations
Differential Diagnosis
Treatment
Capillary Malformations
Venous Malformations
Lymphatic Malformations
Arteriovenous Malformations
Combined Malformations
Acknowledgment
References
Chapter 105: Ulcers
Abstract
Keywords:
Chapter Contents
Introduction
Venous Ulcers
Pathogenesis
Clinical Features
Laboratory Evaluation
Pathology
Differential Diagnosis
Treatment
Moisture and occlusion
Debridement
Wound dressings
Management of wound infection
Adjuncts to wound care
Compression
Manual lymph drainage
Topical negative pressure therapy
Skin substitutes
Topical and oral medications
Lymphedema
Arterial Ulcers
Pathogenesis
Clinical Manifestations
Laboratory Evaluation
Differential Diagnosis
Treatment
Diabetic (and Neuropathic) Ulcers
Pathogenesis
Clinical Features
Laboratory Evaluation
Treatment
Prevention
Ulcers Due to Physical Factors
Pathogenesis
Clinical Features
Pathology
Treatment
Other Causes of Skin Ulceration
Diffuse Dermal Angiomatosis
Hematologic Disorders
Tropical Ulcers
Additional Causes
References
Chapter 106: Other Vascular Disorders
Abstract
Keywords:
Chapter Contents
Introduction
Livedo Reticularis
Introduction
History
Pathogenesis
Clinical Features
Congenital livedo reticularis
Cutis marmorata telangiectatica congenita
Acquired livedo reticularis
Livedo reticularis without systemic associations
Physiologic livedo reticularis/cutis marmorata
Primary/idiopathic livedo reticularis
Livedo reticularis secondary to systemic disease
Livedo reticularis due to vasospasm
Livedo reticularis due to vessel wall pathology
Livedo reticularis due to intraluminal pathology
Other causes of livedo reticularis
Differential Diagnosis
Pathology
Treatment
Flushing
Introduction
Pathogenesis
Clinical Features
Differential Diagnosis
Treatment
Erythromelalgia
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Telangiectasias
Introduction
Spider Telangiectasia
Generalized Essential Telangiectasia
Cutaneous Collagenous Vasculopathy
Unilateral Nevoid Telangiectasia
Angioma Serpiginosum
Hereditary Hemorrhagic Telangiectasia
Ataxia–Telangiectasia
Venous Lakes
Nevus Anemicus
Angiospastic Macules (Bier Spots)
References
Section 18: Neoplasms of the Skin
Chapter 107: Principles of Tumor Biology and Pathogenesis of BCCs and SCCs
Abstract
Keywords:
Chapter Contents
Introduction
Carcinogenesis
The Cell Cycle
Cancer Genes – Oncogenes and Tumor Suppressor Genes
Apoptosis
Limitless Replicative Potential
Angiogenesis
Invasion/Metastasis
Structure and Function of p53
Background
Regulation of the TP53 Gene and p53 Protein
The Cellular Response to DNA Damage
Mutated TP53
Structure and Function of Patched
Background
PTCH1 Gene
PTCH1 Protein Product (Patched)
Normal Function of Patched
Regulation of Patched
PTCH1 Mutations
PTCH2 and SUFU Mutations
Pathogenesis of BCC
Tumor progression
Cancer
Metastasis
Pathogenesis of SCC
Precursors
Cancer
Summary
Related Diseases
Adnexal Tumors
Keratoacanthoma
Xeroderma Pigmentosum
Li–Fraumeni Syndrome
Basal Cell Nevus Syndrome
Bazex Syndrome
References
Chapter 108: Actinic Keratosis, Basal Cell Carcinoma, and Squamous Cell Carcinoma
Abstract
Keywords:
Chapter Contents
Introduction
History
Epidemiology
Pathogenesis
Risk Factors (Table 108.2)
Environmental Exposures
Ultraviolet radiation
Indoor tanning usage
Therapeutic UVR exposure
Ionizing radiation
Occupational risk factors
Chemical exposures
Human Papillomavirus Infection
Immunosuppression
Organ transplantation
Immunosuppressive drugs
HIV infection
Other Risk Factors, Including BRAF Inhibitors
Genetic Risk Factors
Genetic predisposition
Genetic syndromes associated with an increased risk of BCCs and/or SCCs
Xeroderma pigmentosum
Oculocutaneous albinism
Epidermodysplasia verruciformis
Dystrophic epidermolysis bullosa
Basal cell nevus syndrome (BCNS)
Bazex–Dupré–Christol syndrome and Rombo syndrome
Risk of Additional Cancers
Actinic Keratosis and Squamous Cell Carcinoma
Clinical Features
Actinic keratosis
Squamous cell carcinoma in situ
Invasive cutaneous squamous cell carcinoma
Keratoacanthoma
Verrucous carcinoma
Lymphoepithelioma-like carcinoma of the skin
In vivo Imaging Methods
Dermoscopy
Reflectance confocal microscopy (RCM)
Pathology
Actinic keratosis
Squamous cell carcinoma in situ
Invasive squamous cell carcinoma
Keratoacanthoma
Verrucous carcinoma
Histopathologic simulators of SCC
Basal Cell Carcinoma
Clinical Features
Nodular basal cell carcinoma
Superficial basal cell carcinoma
Morpheaform basal cell carcinoma
Fibroepithelial basal cell carcinoma (fibroepithelioma of Pinkus)
Additional histopathologic subtypes of BCC
Basosquamous carcinoma
Micronodular basal cell carcinoma
In vivo Imaging Methods
Dermoscopy
Reflectance confocal microscopy (RCM)
Pathology
Histopathologic simulators of BCC
Treatment
Evaluation and Risk Assessment
Surgical and Destructive Procedures
Standard excision
Curettage with electrodesiccation
Curettage alone
Mohs micrographic surgery
Radiation therapy
Cryosurgery
Photodynamic therapy
Laser
Medical Treatment
Combination therapy
Metastatic NMSC
Prophylaxis
Reduction of immunosuppression in high-risk SCC
Prevention and Early Detection
References
Chapter 109: Benign Epidermal Tumors and Proliferations
Abstract
Keywords:
Chapters Contents
Solar Lentigo
Epidemiology
Pathogenesis
Clinical Features
Associated Diseases and Special Forms
Pathology
Differential Diagnosis
Treatment
Seborrheic Keratosis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Seborrheic Keratosis and Malignancy
Pathology
Differential Diagnosis
Treatment
Lichenoid Keratosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Dermatosis Papulosa Nigra
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Stucco Keratosis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Porokeratosis
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Flegel Disease
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Acrokeratosis Verruciformis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Cutaneous Horn
Clinical Features
Pathology
Treatment
Clear Cell Acanthoma
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Inverted Follicular Keratosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Warty Dyskeratoma
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Acantholytic (Dyskeratotic) Acanthoma
Epidermolytic Acanthoma
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Large Cell Acanthoma
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Epidermal Nevus
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Inflammatory Linear Verrucous Epidermal Nevus
Introduction
History
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Nevus Comedonicus
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Acanthosis Nigricans
Nevoid Hyperkeratosis of the Nipple and Areola
Confluent and Reticulated Papillomatosis
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Clear Cell Papulosis
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Acknowledgment
References
Chapter 110: Cysts
Abstract
Keywords:
Chapter Contents
Introduction
Cysts With a Lining of Stratified Squamous Epithelium
Epidermoid Cyst
Pathology
Treatment
Dilated Pore of Winer
Pathology
Differential diagnosis
Treatment
Pilar Sheath Acanthoma
Treatment
Milium
Pathology
Treatment
Tricholemmal Cyst
Pathology
Treatment
Proliferating Tricholemmal Cyst
Pathology
Treatment
Proliferating Epidermoid Cyst
Pathology
Treatment
Vellus Hair Cysts
Pathology
Treatment
Steatocystoma
Pathology
Treatment
Cutaneous Keratocyst
Pathology
Follicular Hybrid Cyst
Pigmented Follicular Cyst
Dermoid Cyst
Pathology
Treatment
Ear Pit
Pathology
Treatment
Pilonidal Cyst
Pathology
Treatment
Cysts Lined With Non-Stratified Squamous Epithelium
Hidrocystoma
Pathology
Treatment
Bronchogenic Cyst
Pathology
Treatment
Thyroglossal Duct Cyst
Pathology
Treatment
Branchial Cleft Cyst
Pathology
Treatment
Cutaneous Ciliated Cyst and Ciliated Cyst of the Vulva
Pathology
Treatment
Median Raphe Cyst
Pathology
Treatment
Omphalomesenteric Duct Cyst
Pathology
Differential diagnosis
Treatment
Urachal Cyst
Pathology
Treatment
Cysts Without an Epithelial Lining
Mucocele
Pathology
Treatment
Digital Mucous Cyst (Pseudocyst)
Pathology
Treatment
Ganglion
Pathology
Treatment
Pseudocyst of the Auricle
Pathology
Treatment
Cutaneous Metaplastic Synovial Cyst (Pseudocyst)
Pathology
Treatment
References
Chapter 111: Adnexal Neoplasms
Abstract
Keywords:
Chapter Contents
Neoplasms and Proliferations with Follicular Differentiation
Follicular and Folliculosebaceous-Apocrine Hamartomas
Hair follicle nevus
Introduction
Clinical features
Pathology
Treatment
Trichofolliculoma
Introduction
Clinical features
Pathology
Treatment
Fibrofolliculoma, perifollicular fibroma, and trichodiscoma
Introduction
Clinical features
Pathology
Treatment
Nevus sebaceus
Introduction
Clinical features
Pathology
Treatment
Mixed tumor (chondroid syringoma)
Introduction
Clinical features
Pathology
Treatment
Neoplasms and Proliferations With Follicular Germinative Differentiation
Trichoepithelioma/trichoblastoma
Introduction
Clinical features
Pathology
Treatment
Desmoplastic trichoblastoma (desmoplastic trichoepithelioma)
Introduction
Clinical features
Pathology
Treatment
Neoplasms and Proliferations With Matrical Differentiation
Pilomatricoma
Introduction
Clinical features
Pathology
Treatment
Pilomatrical carcinoma
Introduction
Clinical features
Pathology
Treatment
Neoplasms and Proliferations With Follicular Sheath (Tricholemmal) Differentiation
Tricholemmoma
Introduction
Clinical features
Pathology
Treatment
Tricholemmal (trichilemmal) carcinoma
Treatment
Neoplasms and Proliferations With Superficial Follicular (Infundibular and Isthmic) Differentiation
Tumor of follicular infundibulum (infundibuloma; isthmicoma)
Introduction
Clinical features
Pathology
Treatment
Trichoadenoma (trichoadenoma of Nikolowski)
Introduction
Clinical features
Pathology
Treatment
Proliferating pilar tumor (proliferating follicular-cystic neoplasm)
Introduction
Clinical features
Pathology
Treatment
Neoplasms and Proliferations With Sebaceous Differentiation
Sebaceous gland hyperplasia
Introduction
Clinical features
Pathology
Treatment
Sebaceous adenoma, sebaceoma, and sebaceous epithelioma
Introduction
Clinical features
Pathology
Treatment
Sebaceous carcinoma
Introduction
Clinical features
Pathology
Treatment
Neoplasms and Proliferations With Apocrine (or Eccrine) Differentiation
Benign Neoplasms and Proliferations With Apocrine (or Eccrine) Ductal and Tubular Differentiation
Syringoma
Introduction
Clinical features
Pathology
Treatment
Poroma
Introduction
Clinical features
Pathology
Treatment
Hidradenoma
Introduction
Clinical features
Pathology
Treatment
Benign Neoplasms With Apocrine Differentiation
Apocrine adenoma
Introduction
Clinical features
Pathology
Treatment
Undifferentiated Neoplasms of Apocrine Lineage
Spiradenoma
Definition
Clinical features
Pathology
Treatment
Cylindroma
Introduction
Clinical features
Pathology
Treatment
Carcinomas of apocrine lineage
Introduction
Clinical features
Pathology
Treatment
Neoplasms and Proliferations With Eccrine Differentiation
Eccrine nevus (hamartoma)
Treatment
Syringoma, poroma, hidradenoma, adnexal adenocarcinoma
Syringofibroadenoma
Papillary adenoma and adenocarcinoma
Introduction
Clinical features
Pathology
Treatment
References
Chapter 112: Benign Melanocytic Neoplasms
Abstract
Keywords:
Chapter Contents
Ephelides
Epidemiology and Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Café-Au-Lait Macules
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Becker Melanosis (Nevus)
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Solar Lentigines
Lentigo Simplex and Mucosal Melanotic Lesions
Epidemiology
Pathogenesis
Clinical Features
Special forms and associated syndromes
Pathology
Differential Diagnosis
Treatment
Dermal Melanocytosis
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Nevus of Ota and Related Conditions
Nevus of Ota
Epidemiology
Pathogenesis
Clinical features
Related conditions
Pathology
Differential diagnosis
Treatment
Melanocytic Nevi
Blue Nevus and Its Variants
Epidemiology
Pathogenesis
Clinical Features
Common blue nevi
Cellular blue nevi
Epithelioid blue nevi
Pigmented epithelioid melanocytoma
Malignant blue nevi (cutaneous melanoma arising in or having features of blue nevus)
Pathology
Differential Diagnosis
Treatment
Common Acquired Melanocytic Nevi
Epidemiology
Pathogenesis
Clinical Features
Melanocytic nevi associated with epidermolysis bullosa
Melanocytic nevi associated with lichen sclerosus
Pathology
Differential Diagnosis
Treatment
Melanocytic Nevi of Genital Skin and Other “Special Sites”
Clinical Features
Pathology
Differential Diagnosis
Melanocytic Nevus of Acral Skin
Clinical Features
Pathology
Spitz (Spindle and Epithelioid Cell) Nevus/Tumor
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Pigmented Spindle Cell Nevus
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Other Melanocytic Nevi With Spindle Cells
Atypical (Dysplastic) Melanocytic Nevus
History
Epidemiology
Clinical Features
Pathology
Differential Diagnosis
Treatment
Congenital Melanocytic Nevus
Introduction
Epidemiology
Pathogenesis
Clinical Features
Neurocutaneous melanosis (melanocytosis)
Melanoma
Additional associated abnormalities
Pathology
Differential Diagnosis
Treatment
Nevus Spilus
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Halo Nevus
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Combined Nevus
Introduction and Epidemiology
Clinical Features
Pathology
Differential Diagnosis and Treatment
Recurrent Melanocytic Nevus
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 113: Melanoma
Abstract
Keywords:
Chapter Contents
Introduction
Molecular Pathogenesis
Cellular Signaling Pathways in Melanoma
MAPK signaling
PI3K signaling
WNT signaling
MC1R–MITF signaling
Host Immune Response to Melanoma
Epidemiology
Risk Factors for Cutaneous Melanoma
Genetic Risk Factors
Phenotypic Risk Factors Reflecting Gene/Environment Interactions
Common melanocytic nevi
Atypical melanocytic nevi
Ephelides and solar lentigines
Environmental Risk Factors
Ultraviolet radiation
Sun protection
Types of Primary Melanomas
Superficial Spreading Melanoma
Nodular Melanoma
Lentigo Maligna Melanoma
Acral Lentiginous Melanoma
Other Melanoma Variants
Amelanotic Melanomas
Melanoma With Features of a Spitz Nevus (“Spitzoid” Melanoma)
Desmoplastic Melanoma
Clear Cell Sarcoma: Melanoma of Soft Parts
Malignant Blue Nevus
Ocular Melanoma
Mucosal Melanoma
Melanoma and Pregnancy
Childhood Melanoma
Diagnosis
Differential Diagnosis
Dermoscopy
Photography
Reflectance Confocal Microscopy (RCM)
Histopathology
Microstaging
Immunohistopathology
Molecular Analysis
Staging
Prognosis
Evaluation of a Patient With Suspected Melanoma
Medical History
Skin Investigation and Clinical Diagnosis
Laboratory Investigations and Imaging
Management
Management of the Primary Melanoma (Stage I and II)
Local Recurrences
Management of Regional Metastatic Melanoma (Stage III)
Elective lymph node dissection and sentinel lymph node biopsy
Skin satellite and in-transit metastases
Clinically identified lymph node metastases
Adjuvant therapy
Management of Distant Metastases (Stage IV)
Surgery
Radiation therapy
Systemic therapy
Molecularly targeted therapy
Immunotherapy
Immune checkpoint blockade
Cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) blockade
Blockade of programmed cell death 1 (PD-1) and its ligand (PD-L1)
High-dose interleukin-2 treatment
Cancer vaccines
Intralesional oncolytic virus therapy
Adoptive cell therapy
Chemotherapy
Melanoma Surveillance
References
Chapter 114: Vascular Neoplasms and Neoplastic-Like Proliferations
Abstract
Keywords:
Chapter Contents
Introduction
Benign Vascular Neoplasms and Reactive Hyperplasias
Intravascular Papillary Endothelial Hyperplasia (PEH)
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Reactive Angioendotheliomatosis
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Angiokeratomas
Introduction
Clinical features
Solitary or multiple angiokeratomas
Angiokeratomas of the scrotum and vulva
Angiokeratoma corporis diffusum
Angiokeratoma of Mibelli
Angiokeratoma circumscriptum
Pathology
Differential diagnosis
Treatment
Targetoid Hemosiderotic Lymphatic Malformation (Hobnail “Hemangioma”)
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Verrucous Venulocapillary Malformation (Verrucous “Hemangioma”)
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis and treatment
Pyogenic Granuloma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Cherry Angioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Tufted Angioma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Glomeruloid Hemangioma
Introduction and history
Clinical features
Pathogenesis
Pathology
Differential diagnosis
Treatment
Microvenular Hemangioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Epithelioid Hemangioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Multifocal Lymphangioendotheliomatosis With Thrombocytopenia
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Sinusoidal Hemangioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Spindle Cell Hemangioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Borderline and Low-Grade Malignant Vascular Neoplasms
Kaposiform Hemangioendothelioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Papillary Intralymphatic Angioendothelioma (PILA)
Introduction and history
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Retiform Hemangioendothelioma (RHE)
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Kaposi Sarcoma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Malignant Vascular Neoplasms
Epithelioid Hemangioendothelioma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Angiosarcoma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Perivascular Neoplasms and Neoplastic-Like Proliferations
Glomus Tumors and Glomuvenous Malformations
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Infantile Hemangiopericytoma
Introduction and history
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
References
Chapter 115: Neural and Neuroendocrine Neoplasms (Other than Neurofibromatosis)
Abstract
Keywords:
Chapter Contents
Classification, Terminology, and Histogenesis
Neuromas
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Schwannoma
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Neurofibroma
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Nerve Sheath Myxoma and Cellular Neurothekeoma
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Granular Cell Tumor
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Perineurioma
Introduction
Malignant Peripheral Nerve Sheath Tumor
Merkel Cell Carcinoma
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Heterotopic Neural Tissue of the Skin
Heterotopic Neuroglial Tissue
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Heterotopic Meningeal Tissue
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Primitive Neuroectodermal Tumors
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
References
Chapter 116: Fibrous and Fibrohistiocytic Proliferations of the Skin and Tendons
Abstract
Keywords:
Chapter Contents
Skin Tags
Clinical Features
Epidemiology
Pathology
Treatment
Cutaneous Angiofibroma
Clinical Features
Pathology
Dermatofibroma
Clinical Features
Pathology
Treatment
Variants of Dermatofibroma
Acral Fibrokeratoma
Clinical Features
Pathology
Superficial Acral Fibromyxoma
Clinical Features
Pathology
Treatment
Sclerotic Fibroma of the Skin
Clinical Features
Pathology
Pleomorphic Fibroma of the Skin
Clinical Features
Pathology
Multinucleate Cell Angiohistiocytoma
Clinical Features
Pathology
Treatment
Dermatomyofibroma
Clinical Features
Pathology
Treatment
Giant Cell Tumor of Tendon Sheath
Clinical Features
Pathology
Treatment
Fibroma of Tendon Sheath
Clinical Features
Pathology
Treatment
Nodular Fasciitis
Clinical Features
Pathology
Treatment
Connective Tissue Nevus
Clinical Features
Pathology
Clinicopathologic Variants
Differential Diagnosis
Infantile Digital Fibroma
Clinical Features
Pathology
Treatment
Infantile Myofibromatosis
Clinical Features
Pathology
Treatment
Cutaneous Adult Myofibroma
Clinical Features
Pathology
Treatment
Calcifying Aponeurotic Fibroma
Clinical Features
Pathology
Treatment
Fibrous Hamartoma of Infancy
Clinical Features
Pathology
Treatment
Fibromatoses
Clinical Features
Pathology
Treatment
Plexiform Fibrohistiocytic Tumor
Clinical Features
Pathology
Treatment
Atypical Fibroxanthoma
Clinical Features
Pathology
Treatment
Dermatofibrosarcoma Protuberans
Clinical Features
Pathology
Treatment
Giant Cell Fibroblastoma
Clinical Features
Pathology
Treatment
Fibrosarcoma
Clinical Features
Pathology
Treatment
Epithelioid Sarcoma
Clinical Features
Pathology
Treatment
References
E-References
Chapter 117: Smooth Muscle, Adipose and Cartilage Neoplasms
Abstract
Keywords
Chapter Contents
Introduction
Tumors of Smooth Muscle
Leiomyoma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Leiomyosarcoma
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Smooth Muscle Hamartoma
Introduction
History
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Tumors of Fat
Lipoma
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Angiolipoma
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Spindle Cell/Pleomorphic Lipoma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Hibernoma
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Nevus Lipomatosus Superficialis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lipoblastoma/Lipoblastomatosis
Introduction
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Liposarcoma/Atypical Lipomatous Tumor
Introduction
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Tumors of Cartilage
Extraskeletal Chondroma
Introduction
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Acknowledgment
References
Chapter 118: Mastocytosis
Abstract
Keywords:
Introduction
History
Epidemiology
Pathogenesis
Clinical Features
Signs and Symptoms
Cutaneous Lesions
Systemic Manifestations
Classification of Mastocytosis
Pathology
Direct Studies
Indirect Studies
Differential Diagnosis
Treatment
References
Chapter 119: B-Cell Lymphomas of the Skin
Abstract
Keywords:
Introduction
History
Epidemiology
Etiology and Pathogenesis
Clinical Features
Primary Cutaneous Follicle Center Lymphoma
Primary Cutaneous Marginal Zone B-Cell Lymphoma (Extranodal Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue - MALT Lymphoma)
Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type
Intravascular Diffuse Large B-Cell Lymphoma
Precursor B Lymphoblastic Lymphoma/Leukemia
Other Cutaneous B-Cell Lymphomas
Pathology
Primary Cutaneous Follicle Center Lymphoma
Primary Cutaneous Marginal Zone B-Cell Lymphoma (Extranodal Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue – MALT Lymphoma)
Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type
Intravascular Diffuse Large B-Cell Lymphoma
Precursor B Lymphoblastic Lymphoma/Leukemia
Other Cutaneous B-Cell Lymphomas
Differential Diagnosis
Treatment
Plasma Cell Dyscrasias, Including Multiple Myeloma
Histopathology
Differential Diagnosis
Cutaneous and Systemic Plasmacytosis
References
Chapter 120: Cutaneous T-Cell Lymphoma
Abstract
Keywords:
Chapter Contents
Introduction
History
The CTCL concept
The concept of primary cutaneous lymphomas
EORTC, WHO and WHO-EORTC classification schemes
Practical Guidelines for Diagnosis, Classification and Staging of CTCL
Diagnosis
Immunophenotyping
T-cell receptor gene rearrangement analysis
Classification
Practical guidelines
Staging
Mycosis Fungoides
Definition
Epidemiology
Pathogenesis
Genetic factors
Environmental factors
Immunologic factors
Clinical features
Pathology
Immunophenotype
Differential diagnosis
Staging systems and staging procedures
Treatment
Skin-directed therapies
Topical corticosteroids
Topical chemotherapy
Radiotherapy
Phototherapy
Systemic therapies (other than chemotherapy)
Interferons
Retinoids
Denileukin diftitox
Histone deacetylase inhibitors
Systemic chemotherapy
Prognosis
Variants of Mycosis Fungoides
Folliculotropic MF
Definition
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Pagetoid Reticulosis
Definition
Epidemiology
Clinical features
Pathology
Immunophenotype
Differential diagnosis
Treatment
Granulomatous Slack Skin
Definition
Epidemiology
Clinical features
Pathology
Treatment
Sézary Syndrome
Definition
Epidemiology
Clinical features
Pathology
Pathogenesis
Differential diagnosis
Treatment
Adult T-Cell Leukemia/Lymphoma
Definition
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Primary Cutaneous CD30-Positive Lymphoproliferative Disorders
Primary Cutaneous Anaplastic Large Cell Lymphoma
Definition
Epidemiology
Clinical features
Pathology
Immunophenotype
Genetic features
Treatment
Lymphomatoid Papulosis
Definition
Pathogenesis
Epidemiology
Clinical features
Pathology
Differential diagnosis
Treatment
Subcutaneous Panniculitis-Like T-Cell Lymphoma
Definition
Epidemiology
Clinical features
Pathology
Immunophenotype
Treatment
Extranodal NK/T-Cell Lymphoma, Nasal Type
Definition
Epidemiology
Clinical features
Pathology
Immunophenotype
Treatment
Primary Cutaneous CD8-Positive Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma
Primary Cutaneous Gamma/Delta T-Cell Lymphoma
Primary Cutaneous CD4-Positive Small/Medium T-Cell Lymphoproliferative Disorder
Primary Cutaneous Peripheral T-Cell Lymphoma, NOS
References
Chapter 121: Other Lymphoproliferative and Myeloproliferative Diseases
Abstract
Keywords:
Chapter Contents
Benign Lymphocytic Infiltrates
Lymphocytic Infiltrate of Jessner
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Cutaneous Lymphoid Hyperplasia (Pseudolymphoma of the Skin)
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Extramedullary Hematopoiesis
Epidemiology
Pathogenesis
Clinical Features
Pathology
Differential Diagnosis
Treatment
Malignant Hematopoietic Infiltrates
Leukemia Cutis
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Cutaneous Hodgkin Lymphoma
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Blastic Plasmacytoid Dendritic Cell Neoplasm
History and epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Angioimmunoblastic T-cell Lymphoma
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
Lymphomatoid Granulomatosis
History
Epidemiology
Pathogenesis
Clinical features
Pathology
Differential diagnosis
Treatment
References
Chapter 122: Cutaneous Metastases
Abstract
Keywords:
Introduction
Epidemiology
Pathogenesis
Clinical Features
Pathology
Treatment and Prognosis
References
Section 19: Medical Therapy
Chapter 123: Public Health and Dermatology
Abstract
Keywords:
Introduction
The Public Health Importance of Dermatology
Role of Epidemiology in Dermatology
Observational Studies
Descriptive Studies
Using Epidemiology to Identify the Cause of Disease
Case–control study
Cohort study
Cross-sectional study
Assessing the Efficacy of a Preventive or Therapeutic Intervention
Clinical Trials
Systematic Reviews
Genetic Epidemiology
Health Services Research
Health Policy Implications of Research
Quality-of-Life Measures
Important Concepts When Designing and Reviewing a Study
Research Question and Design
Methods
Results
Conclusions
Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) and the Consolidated Standards for Reporting Trials (CONSORT)
Opportunities for Research in Dermato-Epidemiology
Acknowledgment
References
Chapter 124: Skin Barrier and Transdermal Drug Delivery
Abstract
Keywords:
124-1 Skin Barrier
Chapter Contents
Stratum Corneum Structure and Organization
Epidermal Metabolism and the Skin Barrier
Biosynthetic Activities
Lamellar Body Secretion
Extracellular Processing
Acidification
Impaired Skin Barrier and Cutaneous Inflammation
References
124-2 Transdermal Drug Delivery
Chapter Contents
Parameters Affecting Skin Permeability
Parameters Controlling Absorption
Role of the Vehicle
Drug Concentration
Partition Coefficient
Regional Variation
Strategies to Enhance Transdermal Drug Delivery
Chemical Enhancement
Biochemical Enhancement
Physical Enhancement
References
Chapter 125: Glucocorticoids
Abstract
Keywords:
Introduction
Pharmacology and Mechanism of Action
Structure and Metabolism
Absorption and Distribution
The Hypothalamic–Pituitary–Adrenal (HPA) Axis
Molecular Mechanisms
Clinical Indications, Dosages and Contraindications
Oral Therapy
Intramuscular Therapy
Intravenous and Pulse Therapy
Topical Therapy
Intralesional Therapy
Contraindications
Major Side Effects
Osteoporosis
Osteonecrosis
Growth Retardation
Myopathy
Cataracts
Metabolic Effects
Cardiovascular Effects
Gastrointestinal Effects
Infection
Obstetric and Gynecologic Effects
Nervous System Effects
Cutaneous Effects
HPA Axis Suppression
Interactions and Dose Alterations
Acknowledgment
References
Appendix:
Chapter 126: Retinoids
Abstract
Keywords:
Introduction
Mechanism of Action
Vitamin A Metabolism
Retinoid Receptors
Retinoids in the Skin
Synthetic Retinoids
Adapalene
Tazarotene
Acitretin
Isotretinoin (13-cis-retinoic acid)
Bexarotene
Alitretinoin (9-cis-retinoic acid)
Retinoic acid metabolism blocking agents
Indications (Table 126.3)
Topical Retinoids
Acne
Psoriasis
Photoaging
Other indications
Systemic Retinoids
Psoriasis
Acne
Cutaneous T-cell lymphoma
Chronic hand eczema
Other “off-label” clinical uses
Ichthyosis
Darier disease
Pityriasis rubra pilaris
Rosacea
Premalignant and malignant skin lesions
Lupus erythematosus
Dosage
Contraindications
Topical Retinoids
Systemic Retinoids
Major Side Effects
Topical Retinoids
Systemic Retinoids
Teratogenicity
Skin and mucous membrane adverse effects
Systemic toxic effects
Bone toxicity
Muscle effects
Central nervous system and psychiatric effects
Ophthalmologic side effects
Other systemic effects
Hypothyroidism
Gastrointestinal side effects
Renal effects
Laboratory abnormalities
Dyslipidemia
Liver toxicity
Hematologic toxicities
Summary
Interactions
Use in Pregnancy and Lactation
References
Chapter 127: Antimicrobial Drugs
Abstract
Keywords:
Antibacterial Agents
Introduction
Topical Antibacterial Agents
Introduction
Topical antibacterial agents used to treat acne vulgaris and rosacea
Mechanisms of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Topical antibacterial agents used to treat superficial infections
Mechanisms of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Other topical antibacterial agents
Topical tetracyclines
Systemic Antibacterial Agents
Penicillins
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Cephalosporins
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Sulfonamides and co-trimoxazole
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Macrolides
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Tetracyclines
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Clindamycin
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Quinolones
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Metronidazole
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Other antibacterial agents used in Gram-positive skin infections
Dapsone
Antifungal Agents
Mechanisms of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Antiviral Agents
Mechanisms of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Pregnancy and lactation
Other antiviral agents
References
Chapter 128: Systemic Immunomodulators
Abstract
Keywords
Chapter Contents
Interferons
Introduction
Pharmacology and Mechanism of Action
Indications, Off-Label Uses, and Dosages (Table 128.3)
Contraindications
Major Side Effects
Cutaneous reactions
Flu-like symptoms
Neurologic and psychiatric effects
Cardiovascular effects
Rhabdomyolysis
Gastrointestinal effects and bone marrow suppression
Other adverse effects
Interactions
Use in Pregnancy
Granulocyte–Macrophage and Granulocyte Colony-Stimulating Factors (GM-CSF and G-CSF)
Targeted Immune Modulators
Introduction
Tumor Necrosis Factor Inhibitors
Mechanism of action
Contraindications
Major side effects (Table 128.6)
Infections
Risk of malignancy
Autoimmunity
Demyelinating diseases
Congestive heart failure
Cutaneous reactions
Vaccination
Etanercept
Introduction
Indications
Dosages
Specific side effects
Interactions
Use in pregnancy
Infliximab
Introduction
Indications
Dosages
Specific side effects
Interactions
Use in pregnancy
Adalimumab
Introduction
Indications
Dosages
Side effects
Interactions
Use in pregnancy
Golimumab
Certolizumab
Interleukin-12/23 and Interleukin-23 Inhibitors
Introduction
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Use in pregnancy
Interleukin-17 Inhibitors
Contraindications
Major side effects
Interactions
Use in pregnancy
Ixekizumab
Mechanism of action
Indications
Dosages
Secukinumab
Mechanism of action
Indications
Dosages
Brodalumab
Mechanism of action
Indications
Dosages
Additional contraindications and major side effects
Dupilumab
Introduction
Mechanism of action
Indications
Dosages
Contraindications
Major side effects
Interactions
Use in pregnancy
Interleukin-1 Inhibitors
Introduction
Contraindications
Major side effects
Interactions
Anakinra
Mechanism of action
Indications
Dosages
Additional contraindications
Additional major side effects
Use in pregnancy
Canakinumab
Mechanism of action
Indications
Dosages
Pregnancy category
Rilonacept
Mechanism of action
Indications
Dosages
Pregnancy category
Rituximab
Introduction
Mechanism of action
Indications
Dosages
Contraindications
Major side effects (see Table 128.6)
Interactions
Use in pregnancy
Omalizumab
Introduction
Mechanism of action
Indications
Dosages
Contraindications
Major side effects (see Table 128.6)
Interactions
Use in pregnancy
Janus Kinase (JAK) Inhibitors
Introduction
Mechanism of action
Dermatologic uses
Dosages
Contraindications
Major side effects
Interactions
Use in pregnancy
Targeted Immune Modulators Under Investigation and Other Targeted Therapies
Intravenous Immune Globulin (IVIg)
Introduction
Mechanism of Action
Indications
Dosages
Contraindications
Major Side Effects
Interactions
Use in Pregnancy
References
Chapter 129: Other Topical Medications
Abstract
Keywords:
Chapter Contents
Introduction
General Principles of Topical Therapy
Quantity of Application
Formulations and Vehicles
Topical Therapy During Pregnancy and Lactation
Topical Therapy in Neonates
Combination and Rotational Topical Therapy
Antipruritic Agents
Keratolytics and Humectants (See Table 129.8)
Skin-Lightening Agents
Hydroquinone
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Monobenzyl Ether of Hydroquinone
Mequinol
Agents That Affect Hair Growth
Minoxidil
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Bimatoprost
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Eflornithine
Immunomodulatory Agents
Imiquimod
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Topical Calcineurin Inhibitors (TCIs)
Introduction, dosages, and indications
Mechanism of action
Contraindications
Major side effects
Use in pregnancy
Chemotherapeutic Agents
5-Fluorouracil
Mechanism of action
Indications and dosages
Side effects
Contraindications and use in pregnancy
Mechlorethamine Hydrochloride
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Carmustine
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Vitamin D Analogues
Mechanism of action
Topical agents and their indications
Side effects
Use in pregnancy
Miscellaneous Agents
Topical Sirolimus (Rapamycin)
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Diclofenac Sodium
Ingenol Mebutate
Tar
Introduction and indications
Dosages
Mechanism of action
Side effects
Use in pregnancy
Anthralin
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Podophyllin and Podophyllotoxin
Introduction, dosages, and indications
Mechanism of action
Side effects
Use in pregnancy
Cantharidin
Introduction, dosages, and indications
Mechanism of action
Side effects
Sulfur
Introduction
Mechanism of action
Side effects
Contraindications
Use in pregnancy
Azelaic Acid
Introduction and indications
Mechanism of action
Dosages
Side effects
Use in pregnancy
Topical Immunotherapy
The Old and the New
References
Chapter 130: Other Systemic Drugs
Abstract
Keywords:
Chapter Contents
Introduction
Antimalarials
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Apremilast
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Azathioprine
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Bleomycin
Clofazimine
Colchicine
Cyclophosphamide
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Cyclosporine
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Dapsone
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Hydroxyurea
Leukotriene (LT) Inhibitors
Methotrexate
Mechanism of Action
Dosages
Major Side Effects
Drug Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Mycophenolate Mofetil and Mycophenolate Sodium
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
Saturated Solution of Potassium Iodide (SSKI)
Sirolimus (Rapamycin) and Tacrolimus
Thalidomide and Its Derivatives
Mechanism of Action
Dosages
Major Side Effects
Indications
Contraindications
Use in Pregnancy and Lactation
Drug Interactions
References
Chapter 131: Drug Interactions
Abstract
Keywords:
Chapter Contents
Introduction
Putting Interactions Into Perspective
Assessment of Risk in the Clinical Outcome of Drug Interactions
Levels of Evidence
Absorption
P-glycoprotein (PGP)
Distribution
Drug Biotransformation
Cytochrome P450 Enzymes
The Confusing World of Drug Interactions
Metabolism
Cytochrome induction
Cytochrome inhibition
How to Minimize the Risk of Drug–Drug Interactions
Allylamine Antifungals
Azole Antifungals
Azathioprine
Colchicine
Cyclosporine/Everolimus/Sirolimus/Tacrolimus
Grapefruit Juice
Herbal Remedies
HMG-CoA Reductase Inhibitors
Macrolide Antibiotics
Quinolone Antibiotics
Pimozide
Pharmacogenetics – Role of Polymorphisms
Cytochrome P450 enzymes
Other drug-metabolizing enzymes
Drug–Cytokine Interactions
Drug–HIV Interactions
Conclusion
References
Chapter 132: Sunscreens and Photoprotection
Abstract
Keywords:
Chapter Contents
Introduction and History
Sun Exposure
Sunscreens
Sunscreen Regulation
Sun Protection Factor
Testing for UVA Protection
Evolution of FDA and International Sunscreen Standards
Mechanisms of Sunscreen Action
Active Ingredients in Sunscreens
Organic (Soluble) Filters
Para-aminobenzoic acid and derivatives
Cinnamates
Salicylates
Benzophenones
Other organic filters
Inorganic (Insoluble) Filters
Other Photoprotective Agents
Sunscreen Benefits
Sunburn
Photoaging
Photoimmunologic Suppression
Keratinocyte Skin Cancer
Cutaneous Melanoma
Photosensitivity Disorders (see Ch. 87)
Sunscreen Safety
Photoprotective Clothing
Photoprotection Strategies and Sunscreen Recommendations
Special Populations
References
Chapter 133: Complementary and Alternative Medicine
Abstract
Keywords:
Chapter Contents
Introduction
Natural Products – Medical Applications
Aloe Vera
Capsaicin
Chamomile
Colloidal Oatmeal
Escharotics
Feverfew
Green Tea
Honey
Horse Chestnut Seed Extract
Indigo Naturalis
Licorice Root
Marigold
Menthol
Prebiotics and Probiotics
Tea Tree Oil
Turmeric
Vitex
Diet-Based Therapies
Traditional Chinese Medicine
Treatment of Dermatologic Diseases
Atopic dermatitis
Psoriasis
Moxibustion, Cupping and Other Treatment Modalities
Homeopathy
Aromatherapy
Mind–Body Medicine
Ayurveda
Conclusion
References
Section 20: Physical Treatment Modalities
Chapter 134: Ultraviolet Therapy
Abstract
Keywords:
Chapter Contents
Introduction
Phototherapy With UVB
Historical Aspects
Principles and Mechanisms
Action Spectrum and Radiation Sources
Treatment Protocols
Phototherapy for Psoriasis
Phototherapy for Cutaneous T-Cell Lymphoma, in Particular Mycosis Fungoides
Phototherapy for Vitiligo
Phototherapy for Atopic Dermatitis
Broadband UVB (BB-UVB) phototherapy
UVA/UVB phototherapy
Narrowband UVB (NB-UVB) phototherapy
Phototherapy for Photodermatoses
Phototherapy for Pityriasis Lichenoides and Lymphomatoid Papulosis
Phototherapy for Seborrheic Dermatitis
Phototherapy for Pruritus
Side Effects of Phototherapy With UVB
Phototherapy With the 308 nm Excimer Laser or Lamp
Phototherapy With UVA1
Side Effects of Phototherapy With UVA1
Photochemotherapy With Psoralens (PUVA)
Historical Aspects
Psoralens
Psoralen photochemistry
Action Spectrum and Light Sources
Photobiologic Effects of PUVA
Treatment Protocols
Oral PUVA
Bath PUVA
Topical PUVA
Initial Treatment (Clearing) Phase
Maintenance Phase
PUVA for Psoriasis
Combination Treatments
Topical combination
Methotrexate, cyclosporine, systemic immunomodulators (“biologics”)
Retinoids
PUVA for Cutaneous T-Cell Lymphoma, in Particular Mycosis Fungoides
PUVA for Vitiligo
Treatment protocols and results
PUVA for Atopic Dermatitis (See Ch. 12)
PUVA for Lichen Planus (See Ch. 11)
PUVA for Graft-Versus-Host Disease
PUVA for Urticaria Pigmentosa (See Ch. 118)
PUVA for Miscellaneous Dermatoses
PUVA for Photodermatoses
Side Effects and Long-Term Hazards of PUVA
Ophthalmologic effects
Laboratory data
Potential long-term risks of PUVA
UVB and PUVA in HIV-Infected Patients
Extracorporeal Photochemotherapy (Photopheresis)
Treatment Protocols
Mechanism of Action
Side Effects
Other Indications
References
Chapter 135: Photodynamic Therapy
Abstract
Keywords:
Chapter Contents
Introduction
History and Development
PDT – Photochemistry, Photobiology, and Mechanism of Action
PDT Photochemistry
Photobiologic Mechanisms of Action
PDT Selectivity
Advantages and Limitations of PDT
Components of PDT
Photosensitizers
Light Sources
Artificial light sources
Natural daylight
Light delivery
PDT Dosimetry
Dermatologic Applications
Oncologic
Actinic keratoses
Basal cell carcinomas
Squamous cell carcinomas
PDT photochemoprevention of AKs and keratinocyte carcinomas
Mycosis fungoides
Extramammary Paget disease
Cutaneous metastases
Non-Oncologic PDT Applications
Acne
Photoaging
Psoriasis
Cutaneous infections
Vascular anomalies
Hypertrophic scars and keloids
Adverse Effects and Complications of PDT
Photosensitivity
Pain
Inflammation
Photosensitizer Allergy
Rare, Unusual or Controversial Reactions
Practice and Techniques
Future Directions
References
Chapter 136: Lasers and Other Energy-Based Technologies – Principles and Skin Interactions
Abstract
Keywords:
Chapter Contents
Introduction
Lasers
Skin Optics
Thermal Interactions
Selective Photothermolysis
Photomechanical Effects
Cooling for Skin Protection
Applications of Laser Principles
Ablative (Vaporizing) Skin Resurfacing
Interactions During Treatment of Vascular Lesions
Interactions During Treatment of Pigmented Lesions and Tattoos
Interactions During Hair Removal
Non-Ablative Skin Rejuvenation
Fractional Photothermolysis
Laser-Induced Optical Breakdown (Plasma Formation)
Laser-Based Diagnostics
Principles of Laser Safety
Non-Laser Energy Sources
Flashlamps (Intense Pulsed Light)
Radiofrequency
Ultrasound
Low Level Light Therapy (LLLT)
Cryolipolysis
Future Directions
References
Chapter 137: Lasers and Other Energy-Based Therapies
Abstract
Keywords:
Chapter Contents
Types of Lasers and Intense Pulsed Light
Pulsed Dye Laser
KTP Laser (Frequency-Doubled Nd:YAG Laser)
Nd:YAG Laser
Intense Pulsed Light
Treatment of Vascular Lesions
Port-Wine Stains
Infantile Hemangiomas
Telangiectasias
Poikiloderma of Civatte
Telangiectasias and Venulectasias of the Leg (”Spider” Leg Veins)
Verrucae
Pyogenic Granulomas
Venous Lakes and Cherry Angiomas
Pain Considerations
Treatment of Tattoos
Treatment of Melanin-Containing Lesions
Ephelides and Lentigines
Benign Melanocytic Nevi
Café-au-Lait Macules and Nevus Spilus
Nevus of Ota and Nevus of Ito
Melasma
Becker Melanosis (Nevus)
Hyperpigmentation and Discoloration: Postinflammatory, Post-Sclerotherapy, and Drug-Induced
Treatment of Striae Distensae
Ablative Lasers: Carbon Dioxide and Er:YAG
Fractionated Lasers
Non-Ablative Fractional Photothermolysis
Ablative Fractional Photothermolysis
Fractionated Radiofrequency Devices
Preoperative Considerations
Anesthesia and Eye Protection
Post-Therapy Considerations, Side Effects and Complications
Other Photorejuvenation Devices
Skin Tightening – Radiofrequency and Intense Focused Ultrasound
Microwaves
Hair Removal
Home-Based Lasers
Treatment of Psoriasis and Vitiligo
Conclusions
References
Chapter 138: Cryosurgery
Abstract
Keywords
Historical Background
Principles of Cryosurgery and Cryobiology
Cryoimmunology
Instruments and Techniques
Preoperative Considerations
Cryoanesthesia
Treatment
Benign Lesions
Premalignant Lesions, Including Actinic Keratoses
Cutaneous Malignancies
Palliative Treatment
Expected Postoperative Events and Care
Complications
Future Trends (Imaging Technology for Monitoring Cryosurgical Outcome)
References
Chapter 139: Radiotherapy
Abstract
Keywords:
Chapter Contents
Basics of Radiotherapy
Indications
Mechanism of Action
Treatment Machines
Terminology
Radiotherapy Doses
Radiation Physics
Low-Energy Photons (Superficial/Orthovoltage) and Common Cutaneous Protocols
Electrons
Brachytherapy
Office-Based Radiotherapy
Advantages of Radiotherapy
Disadvantages of Radiotherapy
Radiotherapy of Benign Skin Disorders
Background
Keloids
Cutaneous Lymphoid Hyperplasia (Cutaneous Pseudolymphoma)
Radiotherapy of in Situ Cutaneous Malignancies
Squamous Cell Carcinoma in Situ (Bowen Disease)
Lentigo Maligna
Radiotherapy of Invasive Carcinomas, Sarcomas, and Lymphomas
Basal Cell Carcinoma
Definitive radiotherapy
Adjuvant (postoperative) radiotherapy
Squamous Cell Carcinoma
Definitive radiotherapy
Keratoacanthomas
Adjuvant (postoperative) radiotherapy
Special clinical situations
SCC of the lip
Lymph node metastases of SCC
Perineural invasion of SCC and BCC
Immunosuppressed hosts
Dose fractionation schedules and fields
Merkel Cell Carcinoma
Angiosarcoma
Kaposi Sarcoma
Cutaneous Lymphomas
Total skin electron beam therapy
Adnexal Carcinomas
Palliation
Locally Advanced Tumors
Metastases
Reactions and Complications
Acute Reactions
Late Reactions
Younger Patients
Cutaneous Diseases Induced by Radiation Therapy
Future Trends
References
Chapter 140: Electrosurgery
Abstract
Keywords:
Chapter Contents
Introduction and Background
Definition
Electrocautery
Historical Aspects
Electrosurgical Devices and Outputs
Terminology: Monopolar, Bipolar, Monoterminal and Biterminal
Indications/Contraindications
Preoperative History and Considerations
Description of Techniques
Electrodesiccation and Electrofulguration
Electrocoagulation
Electrosection (Cutting)
Variations/Unusual Situations
Cardiac Pacemakers and ICDs
Postoperative Care
Complications
Fire
Thermoelectric Burns
Microorganism Transmission
Surgical Smoke
Electroepilation
Electrolysis vs Thermolysis
Side Effects
Iontophoresis
Future Trends
References
Section 21: Surgery
Chapter 141: Biology of Wound Healing
Abstract
Keywords:
Chapter Contents
Introduction
Regeneration Versus Repair
Effect of Immune Response on Wound Healing
Mechanisms of Tissue Repair and Regeneration Are Evolutionarily Conserved
Amphibians
Zebrafish
Mammals
Clinical Implications
Impact of Wound Depth on Wound Healing
Primary Versus Second Intention Healing
Skin Repair – Cellular and Molecular Aspects
Three Phases of Wound Healing
Cellular Components
Keratinocytes
Endothelial cells
Leukocytes and platelets
Polymorphonuclear leukocytes and platelets
Blood monocytes and tissue macrophages
T cells
Mast cells
Fibroblasts
Stem cells
Growth Factors and Their Receptors
Extracellular Matrix
Integrins
Proteases (Proteinases)
Impaired Wound Healing – Risk Factors
Wound Healing and Aging
Therapeutic Options for Chronic Wounds
Wound Dressings
Growth Factors
Cell- and Tissue-Based Therapies
Future Directions
Stem Cells
Molecular Modeling of Growth Factors
Biomaterials and Tissue Engineering
References
Chapter 142: Surgical Anatomy of the Head and Neck
Abstract
Keywords:
Chapter Contents
Topographic Anatomy of the Head and Neck
Skin Tension Lines
Cosmetic Subunits
Free Margins
The Superficial Musculoaponeurotic System
Muscles of Facial Expression
Vascular Anatomy
The Facial Nerve
Temporal Branch
Zygomatic and Buccal Branches
Marginal Mandibular Branch
Cervical Branch
Sensory Innervation of the Head and Neck
Lymphatic Drainage of the Head and Neck
References
Chapter 143: Anesthesia
Abstract
Keywords:
Introduction
Discussion
Physiology and Structure
Pharmacology
Additions to Local Anesthetics
Epinephrine
Sodium bicarbonate
Hyaluronidase
Anesthetic Mixtures
Side Effects
Vasovagal reactions
Allergic reactions
Local side effects
Overdosage
Topical Anesthetics
Skin
Mucous membranes
Cryoanesthesia
Anesthetic Injection Techniques
Local infiltration
Field block anesthesia
Tumescent anesthesia
Nerve Block Techniques
Facial nerve blocks
Digital nerve blocks
Penile nerve block
Hand nerve block
Foot nerve block
Higher Levels of Anesthesia
References
Chapter 144: Wound Closure Materials and Instruments
Abstract
Keywords:
Introduction
Discussion
Sutures
Types of Suture Material
Absorbable Sutures
Surgical gut
Polyglycolic acid
Polyglactin 910
Polydioxanone
Polyglyconate
Poliglecaprone 25
Glycomer 631 and polyglytone 6211
Non-Absorbable Sutures
Silk
Nylon
Polypropylene
Polyester
Polybutester
Suture Selection
Complications
Needles
Instruments
Curettes
Scalpels
Needle holders
Scissors
Forceps
Hemostats
Skin hooks
Special Instruments
Antiseptics and Sterilization
References
Chapter 145: Dressings
Abstract
Keywords:
Chapter Contents
Introduction
Moist Healing Environment
Role of Debridement
History of Wound Dressings
Functions of a Wound Dressing
Traditional Wound Dressings
First and Second Intention Healing
Wound Cleansers
Topical Antimicrobials and Dressings That Contain Antimicrobial Agents
Moisture-Retentive Dressings
Films
Advantages/disadvantages
Foams
Advantages/disadvantages
Hydrogels
Advantages/disadvantages
Alginates
Advantages/disadvantages
Gelling fibers (Hydrofibers)
Hydrocolloids
Advantages/disadvantages
Composites
Dressings That Reduce Wound Protease Levels
Advanced Wound Therapies
Topical Growth Factors
Tissue-Engineered Skin Equivalents or Skin Substitutes
Epidermal grafts
Dermal replacements
Xenogeneic
Allogeneic
Composite grafts
Negative Pressure Wound Therapy (NPWT)
Hyperbaric Oxygen Therapy (HBOT)
Compression Therapy
Types of Compression
Compression stockings
Compression bandages
Short stretch (inelastic) versus long stretch (elastic) bandages
Compression devices
Future Directions
References
E-References
Chapter 146: Biopsy Techniques and Basic Excisions
Abstract
Keywords:
Chapter Contents
Introduction
Key Concepts
Site Selection
Biopsy Technique Selection
Specimen Handling
Patient Preparation
Site Preparation and Anesthesia
Hemostasis
Wound Closure
Indications/Contraindications
Preoperative History and Considerations
Techniques
Curettage
Snip or Scissors Biopsy
Shave Biopsy and Saucerization Biopsy
Punch Biopsy
Incisional Biopsy
Excisional Biopsy (Excision in toto)
Cutaneous Cone (Dog-Ear) Repair
Variations/Unusual Situations
Postoperative Care
Complications
Future Trends
References
Chapter 147: Flaps
Abstract
Keywords:
Introduction
Background
Fundamentals of Flap Design and Suturing Technique
Flap Movement Characteristics
Burow’s triangle displacement flaps
Defect reconfiguration flaps
Tissue reorientation flaps
Tissue importation flaps
Description of Technique
Burow’s Triangle Displacement Flaps
Single tangent advancement flaps (STAFs) and rotation flaps
Double tangent advancement flaps (DTAFs)
Site-Specific Variations of Burow’s Triangle Displacement Flaps
Helical advancement flaps
Dorsal nasal flap
Other Site-Specific Flaps
Alar rotation flap (ARF) and spiral rotation flap (SRF)
Wedge closure of the lip
Defect Reconfiguration (Island Pedicle) Flaps
Island pedicle flap variations: pincer island pedicle flap
Tissue Reorientation Flaps
Rhombic transposition flap
Bilobed transposition flap
Trilobed flap and rhombic flap with Z-plasty
Nasolabial transposition flap
Tissue Importation Flaps
Forehead flaps
Nasolabial interpolation flap
Retroauricular flap
Spear’s flap
Tunneled flaps
Combination Flaps
Postoperative Care
Complications
References
Chapter 148: Grafts
Abstract
Keywords:
Chapter Contents
Introduction and Background
Wound Healing Considerations
Full-Thickness Skin Grafts
Indications/Contraindications
Preoperative History and Donor Site Considerations
Description of Technique
Securing the Graft
Postoperative Care
Variations/Unusual Situations
Purse-string suture
Burow’s grafts
Deep nasal defects
Postoperative Complications
Future Directions
Split-Thickness Skin Grafts
Indications/Contraindications
Preoperative History and Donor Site Considerations
Description of Grafting Techniques
Securing the Graft
Donor Site Care
Variations/Unusual Situations
Postoperative Care
Complications
Future Directions
Composite Grafts
Indications/Contraindications
Preoperative History and Considerations
Donor Site Considerations for Composite Grafts
Description of Technique
Donor Site Closure
Variations/Unusual Situations
Postoperative Care
Complications
Free Cartilage Grafts
Indications/Contraindications
Nasal Ala
Nasal Sidewall and Tip
Ear
Preoperative History and Donor Site Considerations
Description of Technique
Nasal ala
Nasal sidewall and tip
Ear
Eyelid
Postoperative Care
Complications
Future Trends
Conclusions
References
Chapter 149: Nail Surgery
Abstract
Keywords:
Chapter Contents
Introduction
Surgical Anatomy of the Nail Unit
Basic Anatomy
Innervation
Vascular Supply
Preoperative Evaluation and Patient Preparation
Anesthesia of the Nail Unit
Instruments for Nail Surgery
Nail Plate Avulsion
Biopsy Techniques
Nail Bed Biopsy
Nail Matrix Biopsy
Lateral Longitudinal Biopsy
Proximal Nail Fold Biopsy
Surgical Procedures for Disorders of the Nail Unit
Acute Paronychia
Chronic Paronychia
Ingrown Toenail
Tumors and Cysts
Fibrokeratoma
Subungual exostosis
Glomus tumor
Onychomatricoma
Digital myxoid cyst (pseudocyst)
Squamous cell carcinoma
Melanoma
Postoperative Care
Complications of Nail Surgery
References
Chapter 150: Mohs Micrographic Surgery
Abstract
Keywords:
Chapter Contents
Introduction
History
Training in Mohs Micrographic Surgery
Indications
Appropriate Use Criteria
Cost-Effective Care
Basal Cell Carcinoma
Squamous Cell Carcinoma
Erythroplasia of Queyrat
Keratoacanthoma
Verrucous Carcinoma
Lentigo Maligna
Melanoma Other Than Lentigo Maligna
Atypical Fibroxanthoma
Dermatofibrosarcoma Protuberans
Merkel Cell Carcinoma
Microcystic Adnexal Carcinoma
Sebaceous Carcinoma
Other Adnexal Carcinomas
Other Tumors and Applications
Contraindications
Preoperative History and Considerations
Technique
Variations/Refinements/Special Situations
Single Section Method
“Slow” Mohs Staged Excision and Geometric Staged Excision (for Melanoma)
Non-Beveled 90° Incision
Wide Excision MMS
Rapid Immunohistochemical Stains
Supplementation With Permanent Sections
Bone Invasion
Challenges and Pitfalls
Reconstruction
Postoperative Care
Complications
Future Trends
References
E-References
Chapter 151: Surgical Complications and Optimizing Outcomes
Abstract
Keywords:
Introduction
Discussion
Preoperative Assessment
Potential for bleeding
Laboratory evaluation
Medications and supplements
Wound healing
Prophylactic antibiotics
Defibrillators and pacemakers
Allergies
Informed consent
Intraoperative Considerations
Contamination
Bleeding and hemostasis
Tissue injury
Tension
Necrosis
Nerve deficits
Unsatisfactory scarring
Postoperative Considerations
Bleeding/hematoma/ecchymosis
Infection
Necrosis
Dehiscence
Wound appearance
Conclusions
References
Section 22: Cosmetic Surgery
Chapter 152: Evaluation of Beauty and the Aging Face
Abstract
Keywords:
Chapter Contents
Introduction
Evaluation of Beauty
Anatomic Basis for the Aging Appearance
Photoaging
Pigmentary System
Volume Changes – The Loss of Subcutaneous Fat
Changes in Facial Musculature
Changes in Underlying Cartilage and Bone
Inherent Loss of Elasticity
Gender Differences
Ethnic Differences
Combination Therapies
References
Chapter 153: Cosmetics and Cosmeceuticals
Abstract
Keywords:
Introduction
Discussion
Skin Care Products
Cleansers
Moisturizers
Astringents
Colored Facial Cosmetics
Facial foundations
Formulation
Application and cutaneous effects
Powders
Facial blushes
Eye shadows
Mascaras
Lipsticks
Facial cosmetics for camouflaging
Hair Care Products
Hair shampoos
Hair conditioners
Safety issues
Nail Care Products
Nail polish
Nail sculptures (artificial nails)
Hydroxy Acid Cosmeceuticals
Alpha-hydroxy acids
Beta-hydroxy acid
Additional Cosmeceuticals
Future Directions
References
Chapter 154: Chemical and Mechanical Skin Resurfacing
Abstract
Keywords:
Chapter Contents
Introduction and History
Preoperative History and Considerations
Indications
Contraindications and Patient Selection
Preoperative and Intraoperative Preparations
Chemical Resurfacing Procedures
Superficial Chemical Peeling
Medium-Depth Chemical Peeling
TCA CROSS
Deep Chemical Peeling
Mechanical Resurfacing Procedures
Microdermabrasion
Manual Dermasanding
Motorized Dermabrasion
Microneedling
Postoperative Care
Complications
Conclusions and Future Trends
References
Chapter 155: Phlebology and Treatment of Leg Veins
Abstract
Keywords:
Chapter Contents
Introduction
Venous Anatomy, Physiology and Pathophysiology
Physical Examination of the Phlebology Patient
Laboratory Evaluation of the Superficial Venous System
Compression in Sclerotherapy and Venous Disease
Sclerosing Solutions
Techniques for Treating Telangiectasias and Reticular Veins
Injection Technique
Treatment Protocol
Lasers and Intense Pulsed Light Sources for Treatment of Telangiectasias
Larger Varicose Veins
Foamed Detergent Solutions
Polidocanol 1% injectable foam (Varithena®)
Spontaneous foaming with room air
Duplex Ultrasonography
Postsclerotherapy Compression
Complications
Postsclerotherapy Pigmentation
Telangiectatic Matting
Ulceration
Systemic Allergic Reaction
Arterial Injection
Miscellaneous Complications
Ambulatory Phlebectomy
Preoperative Considerations
Anesthesia and Intraoperative Materials for Ambulatory Phlebectomy
Ambulatory Phlebectomy Technique
Postoperative Considerations for Ambulatory Phlebectomy
Complications of Ambulatory Phlebectomy
Targeting the Saphenofemoral Junction
Endovenous Laser Ablation
Endovenous Radiofrequency Closure (Ablation)
Combination With Ambulatory Phlebectomy
Comparative Clinical Trials
Other Endovenous Procedures for the Treatment of Saphenofemoral Junction Incompetence
Conclusions
References
Chapter 156: Body Contouring
Abstract
Keywords:
Chapter Contents
Introduction and Background
Body Contouring Consultation
Liposuction Procedure
Indications and Contraindications
The Preoperative Visit
Description of Techniques
Tumescent anesthesia
Liposuction surgical technique
Liposuction Instrumentation
Liposuction Devices
Ultrasound-assisted
Power-assisted
Laser-assisted
Water-assisted
Manual liposuction versus device-assisted liposuction
Liposuction Variations
Non-Cosmetic Liposuction
Postoperative Care
Complications of Liposuction
Non-Invasive Body Contouring
Injection Lipolysis
Low-Level Light Therapy
High-Intensity Focused Ultrasound
Non-Thermal Focused Ultrasound
Cryolipolysis
Radiofrequency
Cellulite
Conclusion
Copyright notice
References
Chapter 157: Hair Restoration
Abstract
Keywords
Introduction
Historical Perspective
Male and Female Pattern Hair Loss (Androgenetic Alopecia)
Pathogenesis
Clinical Features
Treatment (Other Than Transplant Surgery)
Medical therapies
Low-level light therapy
Hair Transplantation
Candidate Selection
Key Concepts
Staff Training for Manual Hair Transplantation
Donor Region
Elliptical donor harvesting
Follicular grafts
Follicular unit extraction (FUE)
Advantages of FUE
Follicular unit extraction (FUE) by robotic systems
Hairline Design and Recipient Site Creation
Anesthesia and Recipient Site Creation
Graft Placement and Postoperative Course
Complications
Corrective Hair Transplant Surgery
Adding follicular unit grafts
Surgical removal of grafts
Laser-assisted removal of large grafts
Hypertrophic or broad scars in the donor region
Hair Transplantation in Scarring Alopecias
Hair Transplantation in African-Americans
Future Trends
References
Chapter 158: Injectable Soft Tissue Augmentation
Abstract
Keywords:
Chapter Contents
Historical Perspective
General Injection Technique
Injectable Soft Tissue Filler Materials
Hyaluronic Acid Derivatives
Poly-L-Lactic Acid
Calcium Hydroxylapatite
Autologous Fibroblasts
Lipotransfer
Silicone
Polymethylmethacrylate (PMMA) Microspheres
Platelet-Rich Plasma
Complications
Conclusion
Copyright notice
References
Chapter 159: Botulinum Toxin
Abstract
Keywords:
Chapter Contents
Introduction
Properties of Botulinum Toxins
Formulations
Clinical differences
Topical BoNT-A
A note about dosing
Cosmetic Use of Botulinum Toxin Type A
Glabellar Frown Lines
Crow’s Feet
Horizontal Forehead Lines
Brow Lift and Shaping
Hypertrophic Orbicularis Oculi
Midface Injections
Vertical Lip Rhytides (Perioral Lines)
Facial Asymmetry: Functional Muscle Imbalance
Mouth Frown
Melomental Folds (Marionette Lines)
Mental Crease
Peau d’Orange (Apple Dumpling) Chin
Masseteric Hypertrophy
Platysmal Bands
Adjunctive Use
Medical Uses of Botulinum Toxin Type A
Hyperhidrosis
Raynaud Phenomenon
Migraine
Depression
Complications
Safety
Future Trends
Summary