I Basic Principles. Contents VII

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1 I Basic Principles 1 Introduction Dermatology and theskin... 2 A. Dermatology... 2 B. Frequency ofskin Diseases... 2 C. Functions of the Skin Embryology and Anatomy Embryology and the Epidermis.. 4 A. Embryology... 4 B. The Epidermis... 4 C. The Basement Membrane Zone Adnexal Structures... 6 A. The Adnexal Glands B. Temperature Control The Dermis A. The Dermis B. Extracellular Matrix Components The Hair, Nails, and Subcutaneous Fat A. Hair Development and Structure.. 10 B. The Hair Cycle C. TypesofHair D. Nail Development and Structure.. 12 E. Subcutaneous Fat Biochemistry Keratin A. Keratinization B. Structural Correlates of the Epidermis C. Patterns of Keratinization Melanin A. Melanocytes B. Melanogenesis C. Defects inmelanocytes D. Skin Color and Type E. Approach topigmented Lesions Physiology Cutaneous Sensation A. Cutaneous Innervation B. The Neuronal Basis of Pruritus C. Sympathetic Nerves Immunology Interaction between Innate and Specific Immunity A. Mechanical Barriers B. Innate Immunity C. Specific Immunity Organs of the Immune System A. Organs of the Immune System Cells of the Immune System: Overview A. Development of Immune Cells B. Antigen-presenting Cells C. Granulocytes and MastCells D. Natural Killer Cells TCells A. T-cell Development and Function. 26 B. Interaction between Antigenpresenting Cells and TCells T-cell Differentiation: T H17 and Regulatory TCells A. T H17 and Regulatory TCells B. T H17 Cells C. Regulatory TCells BCells A. Development and Function of BCells Important Mediators of the Immune System A. Cytokines B. Chemokines C. Surface Molecules Intolerance Reactions A. Immediate Reaction (Type I) B. Cytotoxic Reaction (Type II) C. Immune-complexReaction (Type III) D. DelayedReaction (Type IV) Type IReaction: Immediate Hypersensitivity A. IgE Production B. EffectorMechanisms: Immediate Phase C. EffectorMechanisms:Late Phase, Persistent Inflammation Type II and Type III Reactions A. Type II Reaction (ImmunoglobulinmediatedCytotoxicity) B. Type III Reaction (Immune-complex Reaction) VII

2 5.11 Type IV Reaction (Delayed-type Hypersensitivity Reaction) A. Type IV Reaction B. CD4+ T-cell-mediatedImmune Reaction C. CD8+ Immune Reaction Genetics Genetics A. Genodermatoses B. Patterns of Inheritance C. Uses ofgenetics II Diagnosis of Dermatologic Diseases 7 Laboratory In-vitro Diagnosis of Allergy A. Definition and Indications B. Test Procedures C. Cellular Allergen Stimulation Test. 48 D. Eosinophilic Cationic Protein and Tryptase Histopathology A. Biopsy Techniques B. Staining Techniques C. Histological Features D. Patterns of Inflammation E. Approach toslides Immunofluorescence and Electron Microscopy A. Types ofexamination B. Technical Tips C. Examples ofuses D. Advanced Techniques E. Electron Microscopy Mycology and Bacteriology A. Mycological Diagnosis B. Bacteriologic Diagnosis In-vivo Allergy Diagnosis Overview of Allergy Testing A. Requirements for Allergy Testing.. 58 B. Exogenous Influences Physical Testing A. Choice oftestmethods B. Physical Urticaria Skin Tests I A. Skin Tests B. Tuberculin Test Skin Tests II A. PatchTesting B. Test Substances C. Special Situations and Test Modifications Imaging Procedures Dermatoscopy A. Dermatoscopy B. Digital Dermatoscopy Sonography A. Sonography B. Other Imaging Procedures III Treatment of Dermatologic Diseases VIII 10 Medical Therapy Principles oftopical Therapy A. Interactions between Medications and Skin B. Vehicles C. Compounding D. Tricks fortopical Prescribing Corticosteroids A. Structure and Potency B. Mechanisms of Action C. Indications and Side Effects Calcineurin Inhibitors, Azathioprine, and Mycophenolate Mofetil A. Calcineurin Inhibitors B. Azathioprine C. Mycophenolate Mofetil Biologicals A. Definition B. Anti-TNF Therapy C. Anti-IL-12/IL-23p40 Therapy D. New Compounds Cytokines, Toll-like Receptor Agonists A. Immunostimulators B. Interleukin C. Interleukin D. Interferons E. Toll-like ReceptorAgonists F. CTLA-4 Antagonists Thalidomide, Chloroquine, and Fumaric Acid Esters A. Thalidomide B. Chloroquine C. Fumaric Acid Esters... 86

3 10.7 Nonsteroidal Anti-inflammatory Agents and Dapsone A. Nonsteroidal Anti-inflammatory Agents B. Dapsone Cytostatic Agents A. IndirectInhibition of DNA Function B. DirectDNA Interactions C. Molecular Therapy D. Topical Use Antibacterial Agents A. Patient BacteriaInteractions B. Site of Action C. Mechanisms of Action and Resistance D. Classes ofantibiotics E. Topical Antibiotics F. Disinfectants AntimycoticAgents A. Inhibitors of Ergosterol Synthesis. 102 B. Pyridones C. Inhibitors of Cell Wall or DNASynthesis D. Polyene Antibiotics Antiviral Agents A. Mechanisms of Action B. Antiviral Agents in HIV/AIDS Antiparasitic Agents A. Acaricides/Insecticides B. Repellents C. Antihelmintic Agents Retinoids Antihistamines and Antipruritic Agents A. Antihistamines B. Antipruritic Agents Keratolyticsand Antiproliferative Agents A. Keratolytics B. Antiproliferative Agents Miscellaneous Topical Agents A. Antiperspirants B. Medications fordisturbances of Pigmentation C. Skin Cleansers and Protective Agents Physical Modes oftherapy Phototherapy A. UltravioletRadiation B. Principles ofphototherapy C. UVBPhototherapy D. UVAand UVA 1 Phototherapy E. Photochemotherapy Photodynamic Therapy, Radiation Therapy, and Cryotherapy A. Photodynamic Therapy B. Radiation Therapy C. Cryotherapy Laser Therapy A. Principles oflaser Therapy B. Nonspecific Coagulation C. Semi-selective Lasers D. Selective Photothermolysis E. Vaporization and Ablation F. Intense Pulsed Light Operative Dermatology General Aspects and Techniques. 126 A. General Aspects B. Operative Techniques Aesthetic Dermatology A. Botulinum Toxin A B. Fillers C. Peeling D. Laser Skin Resurfacing E. Dermabrasion F. LipAugmentation G. Lifting H. Thermal Lifting I. Liposuction J. CosmeticVein Surgery K. Plastic-reconstructive Surgery IV Dermatologic Diseases 13 Dermatologic Examination History and Symptoms A. History B. Symptoms C. Signs (Physical Findings) Types oflesions A. Primary Lesions B. Secondary Lesions C. Features of Lesions IX

4 13.3 Erythroderma A. Definition B. Pathophysiology C. Etiology D. Clinical Features E. Diagnostic Approach F. Therapy Inflammatory Diseases of the Epidermis Dermatitisand Eczema A. Epidemiology B. Clinical Features C. Histopathology D. Differential Diagnosis E. Therapy Allergic Contact Dermatitis A. Definition B. Epidemiology C. Pathogenesis D. Clinical Features E. Diagnosis and Therapy Irritant Contact Dermatitis and Other Types ofdermatitis A. Definition B. Epidemiology C. Pathogenesis D. Clinical Features E. Therapy F. Other Types ofdermatitis Atopic Dermatitis, Allergic Rhinitis, and Asthma A. Atopic Dermatitis B. Allergic Rhinoconjunctivitis C. Asthma Psoriasis A. Epidemiology and Subtypes B. Pathogenesis C. Histopathology D. Clinical Features E. Course F. Therapy Seborrheic Dermatitis and Reactive Arthritis A. Seborrheic Dermatitis B. Reactive Arthritis Pityriasis Lichenoides, Pityriasis Rosea, and Pityriasis Rubra Pilaris 160 A. Pityriasis Lichenoides B. Pityriasis Rosea C. Pityriasis RubraPilaris Lichen Planus A. Pathogenesis B. Clinical Features C. Histology D. Differential Diagnosis E. Course F. Therapy G. Variants Graft Versus Host Disease A. Pathogenesis B. Clinical Features C. Histopathology D. Course E. Therapy Pruritus and Prurigo A. Pruritus B. LocalizedPruritus C. Prurigo D. Therapy Pemphigus A. Epidemiology B. Pathogenesis C. Clinical Features D. Histopathology and Immunofluorescence E. Course F. Therapy Inflammatory Diseases of the Dermal Epidermal Junction Pemphigoid Diseases and Dermatitis Herpetiformis A. Pathogenesis B. Clinical Features C. Histopathology and Immunofluorescence D. Course E. Therapy F. Dermatitis Herpetiformis Genetic Diseases of the Dermal Epidermal Junction Epidermolysis Bullosa A. Definition B. Pathogenesis C. Clinical Features D. Therapy X

5 17 Inflammatory Diseases of the Dermis Urticaria, Angioedema, and Anaphylaxis A. Pathogenesis B. Clinical Features C. Histopathology D. Diagnostic Approach E. Therapy Erythema Multiforme, Figurate Erythemas, and Nodose Erythemas A. Erythema Multiforme B. Figurate Erythemas C. Nodose Erythemas Dermatomyositis A. Pathogenesis B. Clinical Features C. Histopathology D. Differential Diagnosis E. Course F. Therapy Systemic Sclerosis, Morphea, and Lichen Sclerosus et Atrophicus 184 A. Systemic Sclerosis B. Morphea C. Lichen Sclerosus et Atrophicus Lupus Erythematosus A. Chronic Cutaneous or Discoid Lupus Erythematosus B. Subacute Cutaneous Lupus Erythematosus C. Systemic Lupus Erythematosus D. Course E. Therapy Diseases with Eosinophilia A. Hypereosinophilic Syndrome B. Eosinophilic Cellulitis C. Eosinophilic Fasciitis D. Granuloma Faciale E. Eosinophilic Pustular Folliculitis Noninfectious Granulomatous Diseases A. Sarcoidosis B. Granuloma Annulare C. Necrobiosis Lipoidica D. Rheumatoid Nodule E. Foreign Body Granuloma Inflammatory Diseases of the Subcutaneous Fat Panniculitis A. Overview B. Clinical Types C. Lipoatrophy and Lipodystrophy Genetic Diseases of the Connective Tissue Disorders ofcollagen and Elastin. 200 A. Ehlers Danlos Syndrome B. Marfan Syndrome C. Cutis Laxa D. Pseudoxanthoma Elasticum Genetic Diseases of the Epidermis Disorders ofkeratinization A. Ichthyoses B. Palmoplantar Keratodermas C. Dyskeratotic-acantholytic Disorders D. Follicular Keratoses E. Porokeratoses F. Erythrokeratodermas Mosaicism A. Mosaicism B. Epigenetic Mosaicism C. Genomic Mosaicism D. Acquired Dermatoses inblaschko Lines Epidermal Nevi A. Epidermal Nevi B. Special Types C. Epidermal Nevus Syndromes D. Solitary Lesions Tumors ofthe Epidermis Benign Epidermal Tumors A. Seborrheic Keratosis B. Acanthosis Nigricans C. Confluent and Reticulated Papillomatosis (Gougerot Carteaud Disease) D. Clear Cell Acanthoma Cysts and Adnexal Tumors A. Cysts B. AdnexalTumors Malignant Adnexal Tumors and Paget Disease A. Malignant Adnexal Tumors B. PagetDisease C. Extramammary Paget Disease XI

6 21.4 Basal Cell Carcinoma A. Basal Cell Carcinoma B. Nevoid Basal Cell Carcinoma Syndrome (Gorlin Goltzsyndrome) Squamous Cell Carcinoma and Metastases A. Carcinomata In Situ B. Squamous Cell Carcinoma C. Variants ofsquamous Cell Carcinoma D. Skin Metastases Tumors ofthe Dermis and Subcutis Benign Tumors offat and Connective Tissue A. Benign Tumors of Fat B. Benign Tumors of Connective Tissue Other Benign Soft-tissue Tumors. 228 A. Leiomyoma B. Rhabdomyoma C. Osteoma D. Chondroma Soft-tissue Sarcomas A. Dermatofibrosarcoma Protuberans B. Other Cutaneous Sarcomas Vascular Malformations A. Capillary Malformations B. Venous Malformations C. Lymphatic Malformations D. Arteriovenous Malformations E. Therapy F. Acroangiodermatitis G. Glomus Tumor Hemangiomas and Other Benign Vascular Tumors A. Infantile Hemangioma B. Pyogenic Granuloma C. Eruptive Angioma Malignant and Other Vascular Tumors A. Kaposi Sarcoma B. Angiosarcoma C. Other Vascular Tumors Neural Tumors A. Benign Neural Tumors B. Merkel Cell Carcinoma Hematopoietic Tumors Cutaneous Lymphomas A. Definition and Classification B. Epidemiology C. Pathogenesis D. Parapsoriasis E. Mycosis Fungoides F. Sézary Syndrome G. Other Cutaneous T-cell Lymphomas H. Cutaneous B-cell Lymphomas I. Pseudolymphoma Mast Cell Disorders A. Introduction B. Mastocytoma C. UrticariaPigmentosa D. Telangiectasia Macularis Eruptive Perstans E. Systemic Mastocytosis Histiocytoses and Leukemia A. Langerhans Cell Disease B. Sinus Histiocytosis with Massive Lymphadenopathy C. Juvenile Xanthogranuloma D. Other Macrophage Disorders E. Leukemia Disorders ofmelanocytes and Pigmentation Vitiligo and Albinism A. Vitiligo B. Albinism C. Piebaldism Melanotic Spots and Melanocytic Nevi A. MelanoticSpots B. Melanocytic Nevi Melanoma A. Definition B. Epidemiology C. Pathogenesis D. Clinical Features E. Histopathology F. Differential Diagnosis G. DiagnosticApproach H. Prognosis I. Therapy XII

7 25 Diseases of the Vascular System Vasculitis and Purpura A. Definition B. Classification C. Large-vessel Vasculitis D. Mid-sized-vessel Vasculitis E. Small-vessel Vasculitis F. LeukocytoclasticVasculitis G. Therapy H. Septic Vasculitis I. Thromboangiitis Obliterans J. Purpura K. Pigmented Purpuric Dermatoses L. Antiphospholipid Syndrome M. Livedo Vasculitis N. Pyoderma Gangrenosum O. Behçet Disease Diseases of Veins A. Anatomy and Physiology B. Chronic Venous Insufficiency C. Thrombophlebitis D. Deep Vein Thrombosis Diseases of Adnexal Structures Acne A. Epidemiology and Pathogenesis B. Clinical Features C. Therapy Rosacea A. Epidemiology and Pathogenesis B. Clinical Features C. Therapy Diseases of the Sweat Glands A. Primary Hyperhidrosis B. Secondary Hyperhidrosis C. Hypohidrosis and Anhidrosis D. Chromhidrosis and Bromhidrosis. 276 E. Inflammatory Diseases Diseases of the Hair A. Alopecia B. Hair ShaftAnomalies C. Hypo- and Hypertrichosis D. Hirsutism Diseases of the Nails A. Changes in the Nail Plate B. Changes in Nail Color C. Genetic Nail Anomalies D. Tumors of Nail Region Cutaneous Signs of Systemic Diseases Amyloidosis and Hyalinosis A. Pathogenesis B. Systemic Amyloidosis C. Cutaneous Amyloidosis D. Hyalinosis Diabetes Mellitus A. Overview B. Cutaneous Infections C. Diabetes-associated Dermatoses D. DiabeticNeurovascular Disease E. Complications of Therapy Endocrine Disorders A. Pituitary Gland B. Thyroid Gland C. ParathyroidGlands D. Adrenal Glands E. Pancreas F. Polyendocrine Diseases Gammopathies and Cryoglobulinemia A. Gammopathy B. Clinical Disorders C. Gammopathy-associatedDiseases Hepatic and Renal Diseases A. Cutaneous Signs of Hepatic Disease B. Cutaneous Signs of Specific Liver Diseases C. Cutaneous Signs of Renal Failure/ Dialysis D. Cutaneous Diseases with Renal Involvement Gastrointestinal Diseases A. Cutaneous Signs of Gastrointestinal Disease B. Cutaneous Signs of Gastrointestinal Malignancy C. Inflammatory Bowel Disease Metabolic Diseases A. Disorders of Lipid Metabolism B. Gout C. Hemochromatosis D. Calcification E. Fabry Disease Neurologic Diseases A. Neuropathic Skin Damage B. Neurocutaneous Diseases Paraneoplastic Disorders A. Cutaneous Signs of Internal Malignancy B. Obligate ParaneoplasticMarkers C. Possible ParaneoplasticMarkers XIII

8 XIV 28 Skin Diseases in Different Stages of Life Skin Diseases in Infancy A. Unique Features of Infantile Skin B. Skin Diseases ininfancy Skin Diseases during Pregnancy A. Physiologic Skin Changes B. Dermatoses ofpregnancy C. Infections in Pregnancy Skin Diseases in the Elderly A. Skin Aging B. Features of Aging Skin C. Diseases ofaging Skin Adverse Drug Reactions Adverse Drug Reactions A. Overview B. Severe Skin Reactions C. Classic Drug Reactions D. Unique Drug Reactions Genodermatoses Neurocutaneous Genodermatoses 310 A. Neurofibromatosis B. Tuberous Sclerosis C. Ataxia-telangiectasia Light-sensitive Genodermatoses A. Porphyria B. Xeroderma Pigmentosum Cancer-associated Genodermatoses A. Cancer-associatedSyndromes Psychocutaneous Diseases Psychocutaneous Diseases A. Primary Psychiatric Disorders B. Psychosomatic Disorders C. Secondary Psychiatric Disorders Viral Diseases Human Papillomaviruses A. Clinical Features B. Therapy Human Herpes Viruses A. Herpes SimplexVirus B. Varicella Zoster Virus C. Epstein BarrVirus D. Cytomegalovirus Virus E. Human Herpes Virus F. Human Herpesvirus Other Viruses A. PoxViruses B. PicornaViruses C. Classic Viral Infections of Childhood D. Other Viral Exanthems Bacterial Diseases Staphylococci and Streptococci A. Impetigo B. Folliculitis, Furuncle, and Carbuncle C. Staphylococcal Scalded Skin Syndrome D. Erysipelas E. Other Deep Infections F. Other Infections G. Toxic ShockSyndromes H. ScarletFever Corynebacteria and Other Gram-positiveBacteria A. Corynebacteria B. Other Gram-positive Bacteria Gram-negative Bacteria and Bites. 334 A. Gram-negative Folliculitis B. Whirlpool Dermatitis C. Gram-negative Toe-webInfection. 334 D. CatScratch Disease E. Yersiniosis F. Clostridia Infections G. Bite Injuries Borrelia and Other Spirochetes A. Lyme Borreliosis B. Other Spirochetal Infections Mycobacteria A. Skin Tuberculosis in Anergic Patients B. Skin Tuberculosis in Patients with Normal Resistance C. Leprosy D. Atypical Mycobacteria Fungal Diseases Dermatophytes A. TineaCapitis B. TineaBarbae C. TineaPedis and Manus D. TineaCorporis and Faciei E. TineaInguinalis F. Tinea Unguium G. Special Variants

9 34.2 Yeasts A. Malassezia B. Candida C. Therapy Subcutaneous and Systemic Mycoses A. Subcutaneous Mycoses B. Systemic Mycoses Parasitic Diseases Protozoa A. Leishmaniasis B. Trichomoniasis C. Amebiasis Worms A. Cutaneous LarvaMigrans B. Cercarial Dermatitis C. Subcutaneuos Dirofilariasis D. Onchocerciasis E. Schistosomiasis Arthropods A. Pediculosis B. Cimicosis C. Pulicosis D. Tungiasis E. Myiasis F. Caterpillar Dermatitis G. Scabies H. Demodicosis I. Accidental Parasitic Mites J. Ticks Sexually Transmitted Diseases Gonorrhea and Chlamydial Infections A. Gonorrhea B. Chlamydial Infections C. Nongonococcal Nonchlamydial Urethritis Syphilis A. Early Syphilis B. Late Syphilis C. Congenital Syphilis D. Syphilis in HIV E. DiagnosticApproach F. Therapy Other Venereal Diseases A. Lymphogranuloma Venereum B. Chancroid C. Granuloma Inguinale HIV/AIDS HIV/AIDS A. Epidemiology B. Pathogenesis C. Clinical Classification D. Viral Infections E. Fungal Infections F. Bacterial Infections G. Cancers H. Dermatoses I. DiagnosticApproach J. Therapy Environmental Diseases Nutritional Disorders A. Nutritional Requirements B. Malnutrition C. Eating Disorders D. Zinc E. Iron F. Copper G. Vitamins Photodermatoses A. Overview B. Acute Toxic Effects C. Chronic Harmful Effects D. Sunlight-induced Skin Diseases E. Phototoxic and Photoallergic Reactions F. Idiopathic Disorders G. Photo-aggravated Diseases Heat, Cold, and Other Agents A. Heat B. Cold C. Other Noxious Agents V Appendix Abbreviations Figure Sources Index XV