W. Kempf ] M. Hantschke ] H. Kutzner ] W. H.C. Burgdorf. Dermatopathology

W. Kempf ] M. Hantschke ] H. Kutzner ] W. H.C. Burgdorf Dermatopathology

W. Kempf M. Hantschke H. Kutzner W. H. C. Burgdorf Dermatopathology With 242 Color Figures 12

Werner Kempf MD Kempf und Pfaltz Histologische Diagnostik Schaffhauserplatz 3 8042 Zürich Switzerland Lecturer and Consultant Physician Department of Dermatology University of Zürich Gloria Str. 31 8091 Zürich Switzerland Markus Hantschke MD Dermatopathology Friedrichshafen Siemens Str. 6/1 88048 Friedrichshafen Germany Heinz Kutzner MD Dermatopathology Friedrichshafen Siemens Str. 6/1 88048 Friedrichshafen Germany Walter H. C. Burgdorf MD Traubinger Str. 45A 82327 Tutzing Germany ISBN 978-3-7985-1839-1 Steinkopff Verlag Bibliographic information published by Die Deutsche Nationalbibliothek Die Deutsche Bibliothek lists this publication in the Deutsche Nationalbibliografie; detailed bibliographic data is available in the Internet at http://dnb.d-nb.de. This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Steinkopff Verlag. Violations are liable for prosecution under the German Copyright Law. Steinkopff Verlag a member of Springer Science+Business Media www.steinkopff.com Steinkopff Verlag 2008 Printed in Germany The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publishers cannot guarantee the accuracy of any information about the application of operative techniques and medications contained in this book. In every individual case the user must check such information by consulting the relevant literature. Medical Editors: Dr. med. Gertrud Volkert, Petra Elster Production: Klemens Schwind Cover design: Erich Kirchner, Heidelberg Typesetting: K+V Fotosatz GmbH, Beerfelden Printing and binding: Stürtz GmbH, Würzburg SPIN 12327249 105/7231 543210 Printedonacid-free paper

We dedicate this book to our families

Preface A basic knowledge of dermatopathology is essential for the diagnosis of both inflammatory and neoplastic skin diseases. Our goal in writing this book is to provide a structured well-illustrated introduction to dermatopathology for beginners, not to replace any of the existing standard textbooks. Since the German version was published in the spring of 2007, many positive comments suggest that we may have come close to achieving this goal. Thus we are especially happy to now be able to offer an English version, designed to reach a new group of readers. We would like to thank Gertrud Volkert MD of Steinkopff Verlag for her support and advice in planning and producing both editions. Michael Hertl MD reviewed the information on the diagnosis of blistering diseases. We have limited ourselves to the most common and important diseases in order to make the initial study of dermatopathology as simple and painless as possible. Each disease is presented in a structured fashion, with clinical description, histologic features, special stains, differential diagnostic considerations and comments accompanied by two representative photomicrographs with all of the important diagnostic features labeled with pointers. We have tried to pick representative sections so that the book is useful for the age-old learning tradition of matching pictures to what one sees under the microscope. Especially in the case of inflammatory skin diseases, the clinicopathologic correlation is often the key to the diagnosis. We have tried to mention essential points in the comments section. Similarly, the differential diagnostic possibilities are considered for each individual disease, leading sometimes to repetition, but hopefully enhanced learning and less page turning. We do read the literature but have deliberately not cited any references. Both the Internet and standard dermatopathology textbooks can provide as much directed reading material as anyone could possibly want. Our true hope for this book is that it succeeds in transferring our fascination and love of dermatopathology to at least a few members of the upcoming generations in our specialty. Summer 2008 Werner Kempf Markus Hantschke Heinz Kutzner Walter H. C. Burgdorf

Contents I Basic Principles 1.1 Principles of diagnosis... 3 1.2 The skin biopsy... 5 1.3 Histopathological techniques... 7 1.3.1 Staining... 7 1.3.2 Immunohistochemical stains... 9 1.3.3 Immunofluorescence studies... 11 1.3.4 Molecular biological procedures... 12 1.4 Dermatopathologic glossary... 15 II Inflammatory and Infectious Dermatoses 2.1 Epidermis Spongiosis, acanthosis and hyperparakeratosis... 19 2.1.1 Dermatitis... 20 2.1.2 Prurigo... 22 2.1.3 Psoriasis... 24 2.1.4 Pustular psoriasis... 26 2.1.5 Pityriasis rosea... 28 2.1.6 Cutaneous fungal infections... 30 2.1.7 Viral papillomas... 32 2.1.8 Molluscum contagiosum... 34 2.2 Epidermis Acantholysis... 37 2.2.1 Darier disease... 38 2.2.2 Hailey-Hailey disease... 40 2.2.3 Herpes virus infections... 42 2.3 Bullous diseases... 45 2.3.1 Pemphigus foliaceus... 46 2.3.2 Pemphigus vulgaris... 48 2.3.3 Bullous pemphigoid... 50 2.3.4 Dermatitis herpetiformis... 52 2.3.5 Porphyria cutanea tarda... 54 2.4 Interface dermatoses... 57 2.4.1 Erythema multiforme... 58 2.4.2 Pityriasis lichenoides... 60 2.4.3 Lichen planus... 62 2.4.4 Lichen sclerosus... 64 2.4.5 Lupus erythematosus... 66 2.4.6 Pigmented purpuric dermatoses... 68

X ] Contents 2.5 Dermis Vascular disorders... 71 2.5.1 Leukocytoclastic vasculitis... 72 2.5.2 Granuloma faciale and erythema elevatum et diutinum... 74 2.5.3 Polyarteritis nodosa... 76 2.5.4 Cryoglobulinemia... 78 2.6 Dermis Granulomatous inflammation... 81 2.6.1 Granuloma annulare... 82 2.6.2 Necrobiosis lipoidica... 84 2.6.3 Sarcoidosis... 86 2.6.4 Foreign body granuloma... 88 2.6.5 Mycobacterial infections... 90 2.6.6 Syphilis... 92 2.6.7 Leishmaniasis... 94 2.7 Dermis Interstitial inflammation... 97 2.7.1 Borreliosis... 98 2.7.2 Morphea... 100 2.8 Dermis Diffuse mixed inflammatory infiltrates... 103 2.8.1 Urticaria... 104 2.8.2 Acute febrile neutrophilic dermatosis... 106 2.8.3 Eosinophilic cellulitis... 108 2.8.4 Arthropod assault reaction... 110 2.9 Dermis Degenerative and metabolic disorders... 113 2.9.1 Chondrodermatitis helicis nodularis chronica... 114 2.9.2 Pseudoxanthoma elasticum... 116 2.9.3 Xanthoma... 118 2.10 Dermis Inflammation of adnexal structures... 121 2.10.1 Folliculitis... 122 2.10.2 Alopecia areata... 124 2.10.3 Lupus erythematosus of scalp... 126 2.10.4 Folliculitis decalvans... 128 2.11 Subcutaneous fat Panniculitis... 131 2.11.1 Erythema nodosum... 132 2.11.2 Lupus panniculitis... 134 2.12 Drug reactions... 136 2.13 Artifactual damage... 140 III Cysts 3.1 Epithelial cysts... 145 3.1.1 Epidermoid cyst... 146 3.1.2 Trichilemmal cyst... 148 3.1.3 Steatocystoma... 150 3.2 Pseudocysts... 153 3.2.1 Digital mucous cyst... 154

Contents ] XI IV Hamartomas and Neoplasms 4.1 Epidermal hamartomas and neoplasms... 159 4.1.1 Epidermal nevus... 160 4.1.2 Nevus sebaceus... 162 4.1.3 Seborrheic keratosis... 164 4.1.4 Clear cell acanthoma... 166 4.1.5 Porokeratosis... 168 4.1.6 Actinic keratosis... 170 4.1.7 Bowen disease... 172 4.1.8 Squamous cell carcinoma... 174 4.1.9 Keratoacanthoma... 176 4.2 Melanocytic lesions... 179 4.2.1 Mucosal melanotic macule... 180 4.2.2 Lentigo simplex... 182 4.2.3 Melanocytic nevi junctional and compound types... 184 4.2.4 Melanocytic nevi dermal and congenital types... 186 4.2.5 Halo nevus... 188 4.2.6 Blue nevus... 190 4.2.7 Dysplastic melanocytic nevus... 192 4.2.8 Spitz nevus... 194 4.2.9 Lentigo maligna and lentigo maligna melanoma... 196 4.2.10 Superficial spreading melanoma... 198 4.2.11 Nodular melanoma... 200 4.2.12 Acrolentiginous melanoma... 202 4.2.13 Desmoplastic melanoma... 204 4.3 Adnexal tumors... 207 4.3.1 Sebaceous hyperplasia... 208 4.3.2 Pilomatricoma... 210 4.3.3 Syringoma... 212 4.3.4 Syringocystadenoma papilliferum... 214 4.3.5 Poroma... 216 4.3.6 Hidradenoma... 218 4.3.7 Spiradenoma... 220 4.3.8 Cylindroma... 222 4.3.9 Paget disease... 224 4.3.10 Trichoblastoma... 226 4.3.11 Desmoplastic trichoepithelioma... 228 4.3.12 Basal cell carcinoma... 230 4.3.13 Fibroepithelioma of Pinkus... 232 4.4 Soft tissue proliferations and neoplasms... 235 4.4.1 Scar... 236 4.4.2 Hypertrophic scar and keloid... 238 4.4.3 Skin tag... 240 4.4.4 Dermatofibroma... 242 4.4.5 Dermatofibrosarcoma protuberans... 244 4.4.6 Atypical fibroxanthoma... 246 4.4.7 Leiomyoma... 248 4.4.8 Nevus lipomatosus superficialis... 250 4.4.9 Lipoma... 252 4.4.10 Neurofibroma... 254 4.4.11 Schwannoma... 256 4.4.12 Merkel cell carcinoma... 258

XII ] Contents 4.5 Vascular tumors... 261 4.5.1 Hemangioma... 262 4.5.2 Pyogenic granuloma... 264 4.5.3 Angiokeratoma... 266 4.5.4 Kaposi sarcoma... 268 4.5.5 Angiosarcoma... 270 4.6 Lymphomas and pseudolymphomas... 273 4.6.1 Mycosis fungoides... 274 4.6.2 Primary cutaneous CD30-positive lymphoproliferative diseases... 276 4.6.3 Primary cutaneous follicle center B-cell lymphoma... 278 4.6.4 Cutaneous B-cell pseudolymphoma... 280 4.7 Histiocytoses and mastocytoses... 283 4.7.1 Langerhans cell histiocytoses... 284 4.7.2 Juvenile xanthogranuloma... 286 4.7.3 Cutaneous mastocytoses... 288 4.8. Cutaneous metastases... 290 Subject Index... 293