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Dermatological Signs of Systemic Disease E-Book

Dermatological Signs of Systemic Disease E-Book

Jeffrey P. Callen | Joseph L. Jorizzo | John J. Zone | Warren Piette | Misha A. Rosenbach | Ruth Ann Vleugels

(2016)

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Book Details

Abstract

The new edition of Dermatological Signs of Systemic Disease helps you identify a full range of common and rare systemic diseases early on so they can be managed as effectively as possible. Formerly titled Dermatological Signs of Internal Disease, it takes an evidence-based approach to diagnosis and treatment, offering dependable clinical recommendations that enable you to attain definitive diagnoses of internal diseases that manifest on the skin.

  • Allows you to attain definitive diagnoses of internal diseases that manifest on the skin.
  • Uses a consistent, user-friendly format for easy reference.
  • Covers hot topics such as lupus erythematosus, dermatomyositis, autoinflammatory diseases, eosinophilic and neutrophilic dermatoses, and psoriasis therapies and co-morbidities.
  • Highlights newer therapies and treatment options for most diseases.
  • Includes over 500 full-color illustrations - 200 new to this edition - that provide the best possible representations of diseases as they appear in the clinic.
  • Features more viewpoints from an expanded team of nationally recognized experts in their respective fields.

Table of Contents

Section Title Page Action Price
Front Cover ES1
IFC i
Dermatological Signs of Systemic Disease ii
Dermatological Signs of Systemic Disease iv
Copyright v
Dedication vi
Contents viii
Preface x
Contributors xii
Acknowledgments xviii
1 - LUPUS ERYTHEMATOSUS 1
CHRONIC CUTANEOUS LUPUSERYTHEMATOSUS 1
Discoid Lupus Erythematosus 2
Hypertrophic Lupus Erythematosus 2
Palmar/Plantar Discoid Lupus Erythematosus 2
Oral Discoid Lupus Erythematosus 2
Tumid Lupus Erythematosus 2
Lupus Panniculitis 4
Chilblains Lupus 5
DLE–SLE Subset 5
SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS 5
NEONATAL LUPUS ERYTHEMATOSUS 7
ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS 7
OTHER CUTANEOUS CHANGES ASSOCIATED WITH LUPUS ERYTHEMATOSUS 8
LABORATORY PHENOMENA IN PATIENTS WITH CUTANEOUS LUPUS ERYTHEMATOSUS 9
TREATMENT OF CUTANEOUS LUPUS ERYTHEMATOSUS 10
2 - DERMATOMYOSITIS 13
DEFINITION AND CLASSIFICATION 13
PATHOGENESIS 13
Muscle Disease 16
Systemic Features 16
AMYOPATHIC DERMATOMYOSITIS 17
MYOSITIS AND MALIGNANCY 18
EVALUATION OF THE PATIENT WITH MYOSITIS 18
COURSE AND TREATMENT 19
3 - SCLERODERMA, RAYNAUD’S PHENOMENON, AND RELATED CONDITIONS 22
SCLERODERMA 22
Morphea (Localized Scleroderma) 22
Clinical Manifestations 22
Diagnosis 24
Differential Diagnosis 24
Management 24
Circumscribed Morphea. 25
Linear Morphea. 25
Generalized Morphea. 25
Systemic Sclerosis 25
Clinical Manifestations 25
Diagnosis 26
Differential Diagnosis 27
Management 28
Skin Manifestations. 28
Systemic Manifestations. 28
EOSINOPHILIC FASCIITIS 28
RAYNAUD’S PHENOMENON AND RELATED DISORDERS 29
MIXED CONNECTIVE TISSUE DISEASE 29
NEPHROGENIC SYSTEMIC FIBROSIS (SEE ALSO CHAPTER 38) 29
4 - VASCULITIS 31
INTRODUCTION 31
NOMENCLATURE AND CLASSIFICATION CRITERIA 31
EPIDEMIOLOGY 31
Spectrum of CV in Systemic Vasculitideswith Predominant Organ InvolvementDifferent from the Skin 33
Takayasu Arteritis 33
Polyarteritis Nodosa 33
ANCA-Associated Vasculitis 34
Immune Complex Small Vessel Vasculitis 34
Spectrum of Cutaneous Vasculitis Associated with Autoimmune Systemic Diseases 35
VASCULITIS VARIANTS 35
DIAGNOSTIC APPROACH 36
TREATMENT 36
5 - Neutrophilic Dermatoses 39
BEHÇET’S DISEASE 39
Clinical Manifestations 39
Pathogenesis 39
Histopathology 40
Treatment 40
Pathogenesis 40
Histopathology and Laboratory Findings 41
Associated Conditions 41
Treatment 41
Pyostomatitis Vegetans 41
Atypical or Bullous Pyoderma Gangrenosum 41
Peristomal Pyoderma Gangrenosum 42
Pathogenesis 42
Histopathology 42
Associated Conditions 43
Treatment 43
Pathogenesis 43
Histopathology 43
Treatment 44
6 - PSORIASIS AND SYSTEMIC DISEASE 45
EPIDEMIOLOGY 45
PATHOGENESIS 45
CLINICAL MANIFESTATIONS 45
DIFFERENTIAL DIAGNOSIS 47
TREATMENT 47
Pathogenesis 49
Clinical Manifestations 49
Differential Diagnosis 50
7 - Other Rheumatologic–Dermatologic Conditions 51
8 - AUTOINFLAMMATORY SYNDROMES 59
INTRODUCTION 59
MOLECULAR PRIMER 59
CRYOPYRIN-ASSOCIATED PERIODIC SYNDROME 59
Pathogenesis 59
Clinical Features 59
Evaluation 59
Treatment 60
SCHNITZLER’S SYNDROME 60
Pathogenesis 60
Clinical Features 60
Evaluation 62
Treatment 62
DEFICIENCY OF THE INTERLEUKIN-1 RECEPTOR ANTAGONIST 62
Pathogenesis 62
Clinical Features 62
Evaluation 63
Treatment 63
TUMOR NECROSIS FACTOR RECEPTOR ASSOCIATED PERIODIC SYNDROME 63
Pathogenesis 63
9 - EOSINOPHIL-ASSOCIATED DISEASES WITH DERMATOLOGIC MANIFESTATIONS 69
CLASSIFICATION 69
PATHOGENESIS 70
HISTOPATHOLOGY 72
DIFFERENTIAL DIAGNOSIS 72
TREATMENT 72
10 - URTICARIA 77
PATHOGENESIS 77
CLASSIFICATION 78
Diseases Related to Urticaria 79
Distinctive Urticarial Syndromes 80
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS 82
PATIENT EVALUATION 83
TREATMENT 83
11 - ERYTHEMA MULTIFORME, STEVENS–JOHNSON SYNDROME, AND TOXIC EPIDERMAL NECROLYSIS 87
12 - PANNICULITIS 93
INTRODUCTION 93
PREDOMINANTLY SEPTALPANNICULITIS 93
Erythema Nodosum 93
Superficial Migratory Thrombophlebitis 96
Pancreatic Panniculitis 96
Alpha-1 Antitrypsin Deficiency Panniculitis 97
Lipoatrophy 97
13 - PRURITUS 99
SKIN SIGNS OF CHRONIC PRURITUS 99
COMMON COMPLICATIONS IN PATIENTS WITH GENERALIZED PRURITUS 99
DIAGNOSIS 99
EXAMINATION 100
INVESTIGATIONS 100
PATHOPHYSIOLOGY OF GENERALIZED PRURITUS 100
END-STAGE RENAL DISEASE PRURITUS 101
PRURITUS OF CHOLESTASIS 101
LYMPHOMAS, LEUKEMIA, AND HEMATOLOGIC DISEASE 102
ENDOCRINE DISEASE 102
PRURITUS OF HUMAN IMMUNODEFICIENCY VIRUS INFECTION 102
NEUROPATHIC ITCH 102
PREGNANCY 102
TREATMENT OF PRURITUS 102
14 - ERYTHRODERMA 104
INTRODUCTION 104
CAUSE AND PATHOGENESIS 104
CLINICAL MANIFESTATIONS 105
EVALUATION 106
HISTOPATHOLOGIC FINDINGS 106
COURSE AND TREATMENT 107
15 - PURPURA 109
PURPURA 109
PATHOGENESIS 109
Simple Hemorrhage 109
Petechial Simple Hemorrhage 109
Intravascular Pressure Spikes. 110
Minimally Inflammatory Microvascular Syndromes. 110
Coagulation Cascade Problems in Hemostasis. 110
Other Intravascular Causes of Hemorrhage. 110
Vascular Causes 110
Inflammatory Causes. 110
Noninflammatory Causes. 112
Extravascular Causes 113
Diascopy 114
Inflammation 114
Bland Occlusion 115
HISTORY AND PHYSICAL FINDINGS 115
HISTOPATHOLOGIC FINDINGS 115
DIFFERENTIAL DIAGNOSIS 115
EVALUATION 116
TREATMENT 116
16 - BULLOUS DISEASES 117
PEMPHIGUS 117
Pathogenesis 118
Classification 119
Differential Diagnosis 121
Patient Evaluation 121
Treatment 122
BULLOUS PEMPHIGOID 122
Pathogenesis 123
Classification 123
Differential Diagnosis 124
Patient Evaluation 125
Treatment 125
EPIDERMOLYSIS BULLOSA ACQUISITA 126
Pathogenesis 126
Classification 126
Differential Diagnosis 126
Patient Evaluation 127
Treatment 127
DERMATITIS HERPETIFORMIS 127
Pathogenesis 127
Clinical Features 127
Differential Diagnosis 128
Patient Evaluation 128
Treatment 128
LINEAR IGA BULLOUS DERMATOSIS 129
Classification 129
Pathogenesis 129
Differential Diagnosis 129
Patient Evaluation 130
Treatment 130
17 - Skin Signs of Internal Malignancy 132
PROLIFERATIVE AND INFLAMMATORYDERMATOSES 131
Acanthosis Nigricans 131
Bazex Syndrome 132
Bullous Dermatoses 132
Dermatomyositis and Other Collagen–Vascular Disorders 133
Eruptive Angiomas, Telangiectases, and Seborrheic Keratoses 134
Erythroderma 134
Figurate Erythemas 134
Hypertrichosis Lanuginosa (Malignant Down) 134
Acquired Ichthyosis 135
Keratoacanthoma 135
Migratory Thrombophlebitis (Trousseau Syndrome) 135
Multicentric Reticulohistiocytosis 135
Cutaneous T-Cell Lymphoma (Mycosis Fungoides) 135
Necrobiotic Xanthogranuloma 135
Paget’s Disease of the Breast and Extramammary Paget’s Disease 136
Pityriasis Rotunda 136
Punctate Keratoses and Arsenical Keratoses of the Palms and Soles 136
Pyoderma Gangrenosum and Other “Neutrophilic” Dermatoses 136
Pachydermoperiostosis 137
Tripe Palms 137
Vitiligo 137
Ectopic Adrenocorticotropic Syndrome 137
Necrolytic Migratory Erythema (Glucagonoma Syndrome) 137
Cowden Disease (OMIM 138
Gardner’s Syndrome (OMIM 138
Hereditary Leiomyomatosis and Renal Cell Cancer (OMIM 138
Multiple Endocrine Neoplasia (OMIM 139
Muir–Torre Syndrome (OMIM 139
Peutz–Jeghers Syndrome (OMIM 139
Von Recklinghausen’s Disease (OMIM 140
CONCLUSIONS 140
18 - DERMATOLOGIC ADVERSE EVENTS OF CANCER THERAPY 141
Nitrogen Mustards 141
Aziridines and Epoxides 143
Alkyl Sulfonates 143
Nitrosoureas 146
Hydrazines and Triazine Derivatives 146
Hydroxyurea 146
Antimetabolite Agents 146
Folate Antagonists 146
Pyrimidine Analogs 146
Purine Analogs 147
Topoisomerase Inhibitors 147
Topoisomerase I Inhibitors 147
Topoisomerase II Inhibitors 148
Vinca Alkaloids 148
Estramustine Phosphate Sodium 148
Proteasome Inhibitors 149
Demethylating Agents 149
Retinoids 149
Arsenicals 149
Targeted Anticancer Therapies 149
Epidermal Growth Factor Receptor (EGFR) Inhibitors 149
Multikinase Inhibitors 151
Angiogenesis Inhibitors 151
BRAF Inhibitors 152
Mammalian Target of Rapamycin Inhibitors 152
Rituximab 152
Immune Checkpoint Inhibitors 153
Other Anticancer Agents 153
Endocrine Agents 153
Selective Estrogen Receptor Modulators (SERMs) 153
Estrogen Receptor Downregulators 153
Aromatase Inhibitors 153
Luteinizing Hormone-Releasing Hormone (LHRH) Agonists 153
Androgens 153
Antiandrogens 153
Megestrol Acetate 153
Somatostatin Analogs 153
Bleomycin 154
Thalidomides 154
CONCLUSIONS 154
19 - METASTATIC DISEASE 155
PATHOGENESIS 155
Appearance 156
DIFFERENTIAL DIAGNOSIS 157
HISTOPATHOLOGIC FINDINGS 157
EVALUATION AND PROGNOSIS 158
THERAPY 158
20 - CUTANEOUS LYMPHOMAS AND CUTANEOUS SIGNS OF SYSTEMIC LYMPHOMAS 159
Sézary Syndrome 162
Primary Cutaneous CD30+ Lymphoproliferative Disorders (pcCD30+LD) 162
Other Cutaneous T-cell Lymphomas 163
Primary Cutaneous B-Cell Lymphomas 163
Cutaneous Follicle Center Lymphoma (cFCL) 165
Cutaneous Marginal Zone Lymphoma (cMZL) 166
Cutaneous Diffuse Large B-cell Lymphoma, Leg-type (cDLBCL-LT) 166
Blastic Plasmacytoid Dendritic Cell Neoplasm 166
SPECIFIC MANIFESTATIONS AND NONSPECIFIC SIGNS OF SYSTEMIC LYMPHOMAS 166
Cutaneous B-cell Chronic Lymphocytic Leukemia (B-CLL) 167
Cutaneous Myelogenous Leukemia (cML) 167
Cutaneous Angioimmunoblastic T-cell Lymphoma (cAITL) 167
Cutaneous Hodgkin’s Lymphoma (HL) 168
Nonspecific Signs of Systemic Lymphomas 168
21 - Dysproteinemias, Plasma Cell Disorders, and Amyloidosis 173
Cold Agglutinin Disease 173
Waldenström’s Macroglobulinemia 173
Cryofibrinogenemia 173
Differential Diagnosis 173
Histopathologic Findings 173
Evaluation 174
Treatment 174
Primary Cutaneous Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue Type (PCMZL-MALT): Formerly Cutaneous Plasmacytoma Plasmacytoma 175
Cutaneous and Systemic Plasmacytosis 175
Necrobiotic Xanthogranuloma with Paraproteinemia 176
Other Xanthomatous Diseases 176
Benign Hypergammaglobulinemic Purpura of Waldenström 176
Scleromyxedema 176
Other Disorders 177
Treatment 177
AMYLOIDOSIS 177
Pathogenesis 177
Reactive or Secondary Amyloidosis 179
Hemodialysis-Related Amyloidosis 179
Senile and Mutant Transthyretin (TTR) Amyloidoses (ATTR) 180
Familial Syndromes of Systemic Amyloidosis 180
Skin-Limited Amyloidoses 180
Differential Diagnosis 181
Subcutaneous Fat Aspiration 181
Evaluation 181
Treatment 182
22 - CUTANEOUS MANIFESTATIONS OF THE HISTIOCYTOSES 183
Clinical Manifestations 183
Differential Diagnosis 184
Histopathologic Findings 184
Evaluation 185
Treatment 185
Clinical Manifestations 185
Differential Diagnosis 188
Evaluation 188
Treatment 188
CLASS III: MALIGNANT HISTIOCYTOSIS 188
23 - VASCULAR NEOPLASMS AND MALFORMATIONS 192
BENIGN VASCULAR TUMORS AND MALFORMATIONS 195
Vascular Tumors 196
Vascular Malformations 196
Angiokeratomas 200
KAPOSI’S SARCOMA 200
Clinical Manifestations 200
Histopathologic Findings 202
Evaluation and Treatment 203
ANGIOSARCOMA 203
24 - Diabetes and the Skin 205
Pathogenesis 205
Treatment 205
25 - Thyroid and the Skin 216
Dermatologic Syndromes Associated with Thyroid Cancer 216
Graves’ Disease 218
Congenital Hypothyroidism 220
MISCELLANEOUS CUTANEOUS DISORDERS AND THE THYROID 221
26 - CUTANEOUS MANIFESTATIONS OF LIPID DISORDERS 222
Primary Hyperlipoproteinemias 222
Hyperchylomicronemia 223
Increased VLDLs 223
Increased LDLs 223
Elevated IDLs 224
Secondary Hyperlipoproteinemias 224
Normolipemic Xanthomatosis 224
Hypolipoproteinemias 224
CUTANEOUS XANTHOMAS 225
Tendinous Xanthomas 225
Tuberous Xanthomas 225
Planar Xanthomas 225
Eruptive Xanthomas 226
TREATMENT 226
27 - Adrenal, Androgen-Related, and Pituitary Disorders 229
Pheochromocytoma 230
Deficient Androgen Activity 232
Adrenogenital Syndromes 232
DISORDERS OF STEROID HORMONE RECEPTORS 232
Hypopituitarism 233
28 - PORPHYRIAS 235
Clinical Manifestations 237
Etiologic Associations 237
Evaluation 239
Differential Diagnosis 239
Treatment 239
ERYTHROPOIETIC PROTOPORPHYRIA AND X-LINKED DOMINANT PROTOPORPHYRIA 240
Clinical Manifestations 241
29 - CUTANEOUS DISEASES ASSOCIATED WITH GASTROINTESTINAL ABNORMALITIES 243
Pseudoxanthoma Elasticum 243
Pathogenesis 243
Presentation 243
Evaluation 244
Differential Diagnosis 244
Treatment 245
Hereditary Hemorrhagic Telangiectasia 245
Pathogenesis 245
Presentation 245
Evaluation 246
Differential Diagnosis 246
Treatment 246
POLYPOSIS SYNDROMES 246
Adenomatous Polyposis Syndromes 246
Pathogenesis 247
Presentation 247
Evaluation 247
Treatment 248
Hamartomatous Polyposis Syndromes 248
MALABSORPTION 248
Acrodermatitis Enteropathica 248
Pathogenesis 248
Presentation 249
30 - HEPATIC DISEASE AND THE SKIN 255
PRIMARY BILIARY CIRRHOSIS 257
HEMOCHROMATOSIS 258
WILSON’S DISEASE 258
CUTANEOUS DISEASE ASSOCIATED WITH VIRAL HEPATITIS 259
31 - VIRAL DISEASES 262
Clinical Manifestations 262
Diagnosis 262
32 - BACTERIAL AND RICKETTSIAL DISEASES 271
33 - FUNGAL DISEASES 277
SPOROTRICHOSIS 278
Clinical Manifestations 278
Diagnosis 278
Treatment 278
OTHER SUBCUTANEOUS MYCOSES 278
SYSTEMIC MYCOSES 279
HISTOPLASMOSIS 279
34 - Protozoal Diseases 285
Treatment 285
Diagnosis 286
Treatment 286
35 - ACQUIRED IMMUNODEFICIENCY SYNDROME AND SEXUALLY TRANSMITTED INFECTIONS 289
Cutaneous Manifestations: Infectious 289
Viral Infections 290
Bacterial Infections 292
Fungal Infections 293
Parasitic/Ectoparasitic Infections 294
Cutaneous Manifestations: Inflammatory 294
Cutaneous Manifestations: Neoplastic 296
Cutaneous Manifestations: Miscellaneous 297
Primary Syphilis 297
Secondary Syphilis 298
Tertiary Syphilis 300
Congenital Syphilis 300
Serology Tests. 300
Syphilis in Association with HIV Infection and AIDS 301
Chancroid 301
Granuloma Inguinale 301
Lymphogranuloma Venereum 302
Disseminated Gonococcal Infection 303
36 - SARCOIDOSIS 305
Intrathoracic Disease 309
Ocular Manifestations 310
Lymph Nodes 310
Musculoskeletal Manifestations 311
Neurosarcoidosis 311
Hepatic Sarcoidosis 311
Endocrine, Metabolic, and Laboratory Abnormalities 311
Cardiac Disease 312
Other Clinical Manifestations of Sarcoidosis 312
Relationship of Cutaneous Disease to Systemic Disease 312
HISTOPATHOLOGIC FINDINGS 312
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS 312
EVALUATION 313
PROGNOSIS 313
TREATMENT 313
37 - CARDIOVASCULAR DISEASES AND THE SKIN 315
CARCINOID SYNDROME 315
MALIGNANT ATROPHIC PAPULOSIS (DEGOS DISEASE) 319
EARLOBE CREASES 319
ANDROGENETIC ALOPECIA 320
HEMOCHROMATOSIS 320
RELAPSING POLYCHONDRITIS 320
EMBOLIC PHENOMENA 320
EHLERS–DANLOS SYNDROME 320
MULTIPLE LENTIGINES (LEOPARD) AND NOONAN SYNDROMES 320
PSEUDOXANTHOMA ELASTICUM 321
PSORIASIS AND HEART DISEASE 321
MYOSITIS 321
38 - RENAL DISEASE AND THE SKIN 323
DERMATOLOGIC MANIFESTATIONS OF SYSTEMIC AND GENETIC DISORDERS CAUSING CHRONIC RENAL DISEASE 323
DERMATOLOGIC MANIFESTATIONS OF UREMIA 323
Alopecia 325
Calcinosis Cutis 325
Calciphylaxis 325
Kyrle Disease (Acquired Perforating Dermatoses) 327
Nail Changes 327
Nephrogenic Systemic Fibrosis 328
Pruritus-Related Skin Changes 328
Pigmentary Alteration 329
Bullous Disease (Porphyria and Pseudoporphyria) 329
Uremic Frost 329
Xerosis 329
DERMATOLOGIC CONDITIONS ASSOCIATED WITH RENAL TRANSPLANTATION 329
39 - CUTANEOUS MANIFESTATIONS OBSERVED IN TRANSPLANT RECIPIENTS 330
Antiviral 330
Herpes Simplex Virus (HSV-1, HSV-2) 330
Varicella-Zoster Virus 331
Cytomegalovirus 332
Human Herpesvirus 6 332
Epstein–Barr Virus and Human Herpesvirus 8 332
Human Papillomavirus 333
Molluscum Contagiosum 334
Nocardia. 334
Mycobacteria 334
Deep Fungal Infections 336
Candida. 336
Aspergillus. 337
Cryptococcus. 337
Dimorphic Fungi 338
Other Opportunistic Mycoses and Parasites 338
NEOPLASMS 338
Squamous Cell and Basal Cell Carcinoma 338
Miscellaneous 341
GRAFT-VERSUS-HOST DISEASE 341
Acute GVHD 341
Chronic GVHD 342
DRUG EFFECTS 343
40 - NEUROCUTANEOUS DISEASE 345
NEUROFIBROMATOSIS (VON RECKLINGHAUSEN DISEASE) 346
Pathogenesis 346
Skin Fold Freckling 347
Neurofibromas 347
Lisch Nodules 348
Central Nervous System Involvement 348
Musculoskeletal Disorders 349
Vascular Disorders 349
Gastrointestinal Disorders 349
Endocrine Disorders 349
Miscellaneous Disorders 349
Differential Diagnosis 349
Patient Evaluation 349
Genetic Testing 350
Treatment 350
TUBEROUS SCLEROSIS COMPLEX (BOURNEVILLE’S DISEASE) 350
Pathogenesis 350
Hypomelanotic Macules (Ash Leaf Macules) 351
Ungual Fibromas (Koenen’s Tumors) 351
Shagreen Patch 352
Miscellaneous Nevoid Lesions 352
Central Nervous System Involvement 352
Renal Involvement 352
Cardiac and Pulmonary Involvement 353
Miscellaneous Systemic Findings 353
Differential Diagnosis 353
Patient Evaluation 353
Genetic Testing 354
Treatment 354
STURGE–WEBER SYNDROME 354
Pathogenesis 354
Clinical Manifestations 355
Evaluation and Treatment 355
COBB SYNDROME 356
ATAXIA–TELANGIECTASIA (LOUIS–BAR SYNDROME) 356
Pathogenesis 356
Clinical Manifestations 356
Treatment 356
Waardenburg Syndrome (OMIM 357
Incontinentia Pigmenti (OMIM 357
Hypomelanosis of Ito (Incontinentia Pigmenti Achromians OMIM 357
Vogt–Koyanagi–Harada Syndrome 358
Epidermal Nevus Syndrome 358
Ichthyosis-Associated Syndromes 358
41 - PREGNANCY 359
Jaundice 360
Vascular Changes 360
Palmar Erythema 360
Spider Angioma 360
Edema 360
Varicosities 360
Cutis Marmorata 360
Glandular Changes 360
Hair Changes 361
Nail Changes 361
Mucous Membrane Changes 361
CUTANEOUS NEOPLASMS AFFECTED BY PREGNANCY 361
Melanocytic Nevus 361
Melanoma 362
Molluscum Fibrosum Gravidarum 362
Neurofibroma 362
Pyogenic Granuloma 362
Atopic Dermatitis 362
Autoimmune Progesterone Dermatitis 363
Impetigo Herpetiformis 363
Mucocutaneous Infections 364
Condylomata Acuminata 364
Herpes Simplex Virus Infection 364
Leprosy 364
Varicella-Zoster Virus Infection 364
Vulvovaginal Candidiasis 364
Lupus Erythematosus 365
Pemphigus 365
Systemic Sclerosis 365
Ehlers–Danlos Syndrome 365
Erythema Nodosum 365
Hereditary Hemorrhagic Telangiectasia, Marfan Syndrome, Tuberous Sclerosis 366
Porphyrias 366
Pseudoxanthoma Elasticum 366
PRURITUS IN PREGNANCY 366
Intrahepatic Cholestasis of Pregnancy 366
SPECIFIC DERMATOSES OF PREGNANCY 367
Pemphigoid Gestationis 367
Polymorphic Eruption of Pregnancy 368
Prurigo of Pregnancy 369
Pruritic Folliculitis of Pregnancy 369
42 - MAST CELL DISEASE 370
CLASSIFICATION OF MAST CELL DISEASE 370
Cutaneous Lesions 372
Systemic Manifestations 373
DIAGNOSIS 373
PROGNOSIS 374
TREATMENT 374
43 - HAIR DISORDERS IN SYSTEMIC DISEASE 377
HAIR DISORDERS IN SYSTEMIC DISEASE 378
ALOPECIA 378
Telogen Effluvium 378
Immediate Anagen Release 378
Delayed Anagen Release 378
Short Anagen 379
Immediate Telogen Release 379
Delayed Telogen Release 379
Telogen Effluvium and Drugs 379
Telogen Effluvium and Androgenetic Alopecia 379
Chronic Telogen Effluvium 379
Diagnosis and Treatment 379
Anagen Arrest 380
Diagnosis and Treatment 381
Androgenetic Alopecia/Hair Miniaturization 382
Diagnosis and Treatment 382
Scarring Alopecia (Cicatricial Alopecia) 382
Diagnosis and Treatment 383
Hair Shaft Disorders 383
Diagnosis and Management 384
EXCESSIVE HAIR 385
Hypertrichosis 385
Diagnosis and Treatment 385
Hirsutism 385
Diagnosis and Treatment 386
44 - NAIL SIGNS OF SYSTEMIC DISEASE 387
Mees Lines 387
Pitting 388
Longitudinal Pigmented Bands (Melanonychia) 388
Brittle Nails 388
Koilonychia 388
Muehrcke Lines, Half-and-Half Nails (Lindsay nails), and Terry Nails 391
Onycholysis 392
Nail Fold Capillary Abnormalities 393
Gastrointestinal System 393
Endocrine System 393
Infectious Diseases 394
Central and Peripheral Nervous Systems 394
Psychiatric Conditions 394
Pulmonary System 394
Renal and Genitourinary Systems 394
Reproductive System 395
Rheumatologic Diseases 395
Nail Changes Associated with Malignancy 396
Miscellaneous 396
45 - ORAL DISEASE 397
CHEILITIS GRANULOMATOSA (MELKERSSON–ROSENTHAL SYNDROME) 397
NECROTIZING ULCERATIVE GINGIVITIS 400
ORAL PIGMENTATION 400
XEROSTOMIA 400
GINGIVAL HYPERPLASIA 401
46 - LEG ULCERS 402
Pathophysiology of Chronic Ulcerations 404
Pathophysiology of Venous Ulceration 406
Pathophysiology of Arterial Ulceration 407
PATIENT HISTORY AND PHYSICAL EXAMINATION FINDINGS 407
History 407
Physical Examination 408
COMMON CAUSES OF ULCERATION 409
Venous Ulcerations 409
Arterial Ulcerations 410
Atypical Wounds 411
Vascular Studies 412
Venous Studies 412
Arterial Studies 414
Biopsy 414
Wound Swabs 420
Radiographic Studies 420
PROGNOSIS 420
GENERAL PRINCIPLES OF WOUND CARE 420
Debridement of Devitalized Tissue 420
Management of Infection 420
Wound Dressings 421
Limb Elevation 422
Compression 422
Elastic Support Stockings 422
Unna Boot 422
Elastic Multilayer Bandaging Systems 422
Skin Grafts 422
Tissue-Engineered Products 422
Pentoxifylline 423
Aspirin 423
Venous Surgery 423
Pain Management 423
Management of Arterial Ulcerations 423
Acknowledgment 423
47 - CUTANEOUS DRUG ERUPTIONS 425
EPIDEMIOLOGY OF CUTANEOUS DRUG REACTIONS 425
DRUG-INDUCED SKIN INJURY 425
APPROACH TO THE PATIENT WITH A SUSPECTED DRUG ERUPTION 425
MECHANISMS OF CUTANEOUS DRUG ERUPTIONS 428
Complex Eruption: Drug-Induced Hypersensitivity Syndrome 430
Complex Urticarial: Serum Sickness-Like Reaction 431
Complex: Acute Generalized Exanthematous Pustulosis 432
Drug-Induced Bullous Pemphigoid 432
Drug-Induced Immunoglobulin-A Bullous Dermatosis 432
Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis 433
Neutrophilic Eccrine Hidradenitis 434
Eruptions Caused by Biologic Treatments 434
Drug-Induced Lupus 434
Vasculitis 434
Other Miscellaneous 435
CONCLUSIONS 435
48 - Principles of Systemic Drug Use 437
Principle 3. “Risk–Risk” Analysis Is Performed by Comparing the Risk(s) of a Given Disease (As Defined Earlier) with the Inheren... 437
Principle 4. It Is Important to Be Aware of a Given Drug’s Official Food and Drug Administration (FDA)-Approved Indications, and... 438
Principle 5. The Priority Sequence of Systemic Drug Choices Should Be Individualized for Each Specific Patient. Factors Such As ... 438
Principle 3. Monitoring for Adverse Effects Associated with Systemic Drugs Used in Dermatology Is Largely Based on Risk Reductio... 439
Principle 4. Virtually All Tests and Examinations to Be Used in the Monitoring Process Should Have a Baseline Determination 440
Principle 5. “Critical Toxicities” Are Defined as Any Drug-Induced Adverse Effect that May Result in Either Loss of Life or Pote... 440
Principle 6. Risk Reduction Can Be Optimized through the Use of Well-Defined Monitoring Guidelines 440
Principle 7. Monitoring Guidelines Are Based on Data from Low-Risk Patients with Normal Test Results. More Frequent Surveillance... 440
Principle 9. Share the Responsibility of Monitoring for Adverse Effects with Other Appropriate Specialists 441
Principle 10. Minimize the Risk of Systemic Drug Therapy through Adjunctive Therapy with Other Systemic Drugs and Topical or Loc... 441
Index 443
A 443
B 444
C 445
D 447
E 447
F 448
G 448
H 449
I 450
J 451
K 451
L 451
M 452
N 453
O 453
P 453
Q 455
R 455
S 456
T 457
U 458
V 458
W 459
X 459
Y 459
Z 459