O. BRAUN-FALCO· G. PLEWIG H.H. WOLFF· W.H.C. BURGDORF

Dermatology

Springer Berlin Heidelberg New York Barcelona Hong Kong London Milano Paris Singapore Tokyo

O. BRAUN-FALCO • G. PLEWIG H.H. WOLFF • W.H.C. BURGDORF

DERMATOLOGY Second, Completely Revised Edition

With 1038 Color Figures and 281 Tables

Springer

Prof. Dr. med. Dr. h.c.mult. OTTO BRAUN-FALCO Emeritus Professor and Chairman Department of Dermatology and Allergology Ludwig Maximilians University FrauenlobstraBe 9 -11

D-80337 Munich, Germany

Prof. Dr. med. GERD PLEWIG Professor and Chairman Department of Dermatology and Allergology Ludwig Maximilians University FrauenlobstraBe 9 -11

D-80337 Munich, Germany

Prof. Dr. med. HELMUT H. WOLFF Professor and Chairman Department of Dermatology and Venerology University of Lubeck Ratzeburger Allee 160 D-23562 Lubeck, Germany

WALTER H.C. BURGDORF, M.D. Clinical Lecturer Department of Dermatology and Allergology Ludwig Maximilians University FrauenlobstraBe 9 -11

D-80337 Munich, Germany

Based on: O. Braun-Falco, G. Plewig, H. H. Wolff: Dermatologie und Venerologie, 4th revised edition. © Springer-Verlag Berlin Heidelberg 1996

Library of Congress Cataloging-in-Publication Data Dermatologie und Venerologie. English, Dermatology / O. Braun-Falco ... [et al.l. - 2nd, completely rev. ed. p. ; cm. Includes bibliographical references and index.

ISBN-13: 978-3-642-97933-0 DOI:IO.1007/ 978-3-642-97931-6

e-ISBN-13: 978-3-642-97931-6

1. Skin - Diseases - Diagnosis. 2. Sexually transmitted diseases - Diagnosis. I. Braun-Falco, Otto. II. Title. [DNLM: 1. Skin Diseases. 2. Sexually Transmitted Diseases. WR 140 04354 2000al RL105 .45413 2000

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Springer-Verlag Berlin Heidelberg New York a member of BertelsmannSpringer Science+ Business Media GmbH © Springer-Verlag Berlin Heidelberg 2000

Softcover reprint of ilie hardcover 2nd edition 2000

The use of general descriptive names, registered names, trademarks, etc. in iliis publication does not imply, even in ilie absence of a specific statement, iliat such names are exempt from ilie relevant protective laws and regulations and ilierefore free for gen­eral use.

Product liability: The publishers guarantee ilie accuracy of any information about dosage and application contained in iliis book. In every individual case ilie user must check such information by consulting ilie relevant literatur.

Production editor: W. Bischoff, Heidelberg Cover design: de'blik Konzept & Gestaltung, Berlin Illustrations: R. Darroll, Hamburg Reproduction of figures: Schneider Repro, Heidelberg Data conversion: Fotosatz-Service Kohler GmbH, Wiirzburg

22/3111 - 5 4 3 2 1 Printed on acid-free paper

Preface

This text book has a long history. The first two German editions of Dermatologie und Vene­rologie were written by Egon Keining and Otto Braun-Falco and appeared in 1961 and 1969. Otto Braun-Falco invited two of his proteges, Gerd Plewig and Helmut H. Wolff, to assist him in writing the third German edition, which was published in 1984. These authors, along with Richard K. Winkelmann, joined together, in 1991, to create the first English edition. The fourth German edition appeared in 1996 and now the second English edition is ready.

This second English edition is quite different from both the fourth German edition and the first English edition. The fourth German edition was used as the framework for an entire­ly new translation; this work is not based on the first English edition. Many new topics have been introduced and much of the material has been reorganized. Two new chapters, on diseases of black skin and operative dermatology, have been added. Every chapter has been checked by at least two of the German authors; most have been revised several times.

This book is designed for a wide variety of readers. Dermatology residents will find it valuable both in their daily work and when reviewing for specialty board examinations. Experienced dermatologists should be able to update their knowledge, as well as refer to the chapters on specific diseases for therapeutic options. Ambitious medical students may want to use our book as an introduction to dermatology, for it will stand them in good stead as they pursue training in other specialties. Finally, for the increasing number of primary care physicians, be they family doctors, pediatricians, internists, gynecologists or emergency room physicians, caring for patients with skin diseases, we hope that this book will be a help­ful source of information that can be used in daily practice.

Dermatology remains a highly visual specialty, and in this respect the major strengths of this book are unchanged. We have concentrated on providing detailed clinical and morpho­logical descriptions and excellent clinical photographs. Many new photographs have been added or have replaced older ones. To be more representative, we have also included photo­graphs of black and Hispanic patients. In addition, all of the illustrations in the book have been redrawn. While a correlation between the clinical findings and the histology remains a cornerstone of dermatologic diagnosis, we have once again not included photomicrographs. Instead, the histological descriptions are designed to orient the reader and not to be exhaus­tive. Many excellent dermatopathology books are available; some are almost as long as this book. We refer the reader to these sources for more detailed information.

The changes since the last English edition have been immense. The pathogenesis of many diseases is better understood because of advances in immunology, molecular biology and molecular genetics. We have tried to distill these advances into a form understandable to the student, resident or practicing physician. In the process, we have undoubtedly oversimpli­fied and introduced mistakes, for which we apologize in advance. Finally, the HIV I AIDS epi­demic has once again made the sexually transmitted diseases and other infectious diseases such as syphilis and tuberculosis critically important, so we have retained the extensive sec­tions on these disorders.

Many friends and colleagues helped with this book. Several colleagues took complete re­sponsibility for a chapter: Roselyn E. Epps and John A. Kenney, Jr., (Chapter 66 Diseases of Black Skin), Hans Wolff and Wolf-Bernhard Schill (Chapter 68 Andrology) and Rainer Rom­pel (Ch<l;pter 72 Operative Dermatology). Other colleagues provided particular input into either new sections or ones that received extensive revision: Wybo Bruinsma (drug reac­tions), Rudolf Happle (genetics and epidermal nevi), Axel Hauschild (malignant mela-

VI Preface

noma), Ulrich Hohenleutner (stump dermatoses), Thomas Jansen (acne and rosacea), Michael Landthaler (lasers), Gerald Messer (bullous and connective tissue diseases), Maja Mockenhaupt (severe skin reactions), Bernhard Ortel (phototherapy equipment), Martin R6cken (immunology), Franziska Rueff (allergy), Christian Sander (malignant lymphoma), Martin Schaller (infectious diseases), Eva-Regina Thoma-Greber (AIDS) and Franz Trautin­ger (phototherapy). Elke Bornh6vd attempted to identify as many original reference cita­tions as possible and helped extensively with checking of other references and responding to copy editing queries. Anette Bonowitz and Tilmann Oppel helped with the index. Other colleagues provided figures, including J urg Hafner, Rebat M. Halder, Michael Landthaler, Juan J. Ochoa, Johannes Ring, Thomas Ruzicka, Robert H. Schosser and Robert Swerlick.

Once again many co-workers from the Department of Dermatology, Ludwig Maximilian University, Munich, reviewed chapters and helped with references. They include: Hans Peter Bertsch, Ursula B6hmer, Susanne Breit, Elke Bornh6vd, Rene Chatelain, Klaus Degitz, Michael Flaig, Kamran Ghoreschi, Marc Heckmann, Thomas Herzinger, Thomas Jansen, Peter Kaudewitz, Gerold Kick, Hans Christian Korting, Christian Kunte, Dagmar Ludolph­Hauser, Gerald Messer, Silke Michelsen, Uwe Neubert, Bettina Prinz, J6rg Prinz, Martin R6cken, Ricardo Romiti, Franziska Rueff, Rudolf Rupee, Arne Sakrauski, Christian Sander, Martin Schaller, Monika -Hildegard Schmid -Wendtner, Kathrin Schuhmann, Eva-Regina Thoma-Greber, Peter Thomas, Beata Trautner, Sabine Werfel, Ralf Wienecke, Hans Wolff, Andreas Wollenberg and Sabine Zenker.

Finally, the following colleagues helped with the fourth German edition, and thus some of their work has been carried over into this translation. Their titles and addresses are given in the German text. They include: Dietrich Abeck, Ulrich Amon, Thomas Bergner, Thomas Bie­ber, Leena Bruckner-Tuderman, Friedemann Enders, Peter Frosch, Gunter Goerz (t), Wolf­gang Hartschuh, Conrad Hauser, Stefan H6dl, Erhard H6lzle, Heiko Iven, Thomas Jansen, Peter Kaudewitz, Martina Kerscher, Peter Kind, Peter Karl Kohl, Hans Christian Korting, Jurgen Kreusch, Thomas Krieg, Wolfgang Kuster, Michael Landthaler, Bodo Melnik, Michael Meurer, Helmut Naher, Uwe Neubert, J6rg Prinz, Bernhard Przybilla, Johannes Ring, Martin R6cken, Thomas Ruzicka, Christian Sander, Martin Schaller, Rudiger Scharf, Wolf­Bernhard Schill, Carl-Georg Schirren, Wilfried Schmeller, Eva-Regina Thoma-Greber and Michael Tronnier.

Many other thanks are also due. Once again Peter Bilek, our clinic photographer in Munich, provided invaluable aid, as did his assistants Claudia Jakobec and Diana Keller­meier. Robert Darroll, a free-lance medical illustrator, executed the new drawings. Claudia Tielkes managed the entire process of transferring material back and forth between authors, colleagues and the publishers without a glitch. Springer-Verlag, and especially Willi Bischoff, was very patient with the slow writing process and provided their usual excellent support in terms of editorial advice, design and production.

We suspect this will be one of the last textbooks of this size to be typed by a single physi­cian, as WB transcribed each chapter, translating and modifying the German text. This approach has resulted in a uniform style and hopefully kept replications and contradictions to a minimum. Finally, we thank a most unusual helper - an aging, red Manx cat named Boris who sat by WB's side for almost all of the computer time required to produce this text. If one can learn by proximity, then he is a feline master of dermatology.

Munich and Lubeck, 2000 OTTO BRAUN-FALCO

GERD PLEWIG

HELMUT H. WOLFF

WALTER H.C. BURGDORF

Contents

Chapter 1

Basic Science and Principles of Dermatologic Diagnosis

Chapter 2

Viral Diseases

Chapter 3 Rickettsial Diseases

Chapter 4 Bacterial Diseases

Chapter 5 Sexually Transmitted Bacterial Diseases

Chapter 6 Protozoan Diseases

Chapter 7 Fungal Diseases

Chapter 8 Diseases Caused by Arthropods

Chapter 9 Diseases Caused by Worms

Chapter 10

Reactions to Medications

Chapter 11

Urticaria, Angioedema and Anaphylaxis

Chapter 12

Dermatitis

Chapter 13 Diseases Caused by Environmental Exposure orTrauma

Chapter 14 Erythemato-Papulo-Squamous Diseases

Chapter 15 Blistering Diseases

1

53

121

l27

245

299

313

359

431

457

521

571

VIII

Chapter 16 Pustular Diseases

Chapter 17 Disorders of Keratinization

Chapter 18 Diseases of Connective Tissue

Chapter 19 Malformations and Genetic Disorders

Chapter 20

Inflammatory Diseases of Cartilage

Chapter 21

Diseases of the Subcutaneous Fat

Chapter 22

Diseases of the Blood Vessels

Chapter 23 Disorders of Hemostasis

Chapter 24 Diseases of the Lymphatics

Chapter 25

Contents

Pruritus, Prurigo, Self-Induced Disease, Psychiatric Disease and Neurologic Disease . . . . . . . . . . . . . . . . . . .

Chapter 26 Disorders of Melanin Pigmentation

Chapter 27 Dyschromias

Chapter 28 Diseases of the Sebaceous Glands

Chapter 29 Diseases of the Apocrine Sweat Glands

Chapter 30

Diseases of the Eccrine Sweat Glands

Chapter 31

Diseases of Hair

Chapter 32 Diseases of the Nails

Chapter 33 Disorders of Lips and Oral Mucosa

709

751

855

861

881

955

1013

1043

1051

1099

1141

Contents

Chapter 34 Diseases of the Male Genitalia

Chapter 35 Diseases ofthe Female External Genitalia

Chapter 36 Skin Diseases of Pregnancy

Chapter 37 Disorders of Lipoprotein and Lipid Metabolism

Chapter 38 The Sphingolipidoses

Chapter 39 Disorders of Amino Acid Metabolism

Chapter 40 Gammopathies

Chapter 41 Amyloidoses

Chapter 42 The Hyalinoses

Chapter 43 Mucinoses

Chapter 44 The Porphyrias

Chapter 45 Cutaneous Calcification

Chapter 46 Disorders of Mineral Metabolism

Chapter 47 Disorders of Purine Metabolism

Chapter 48 Nutritional, Metabolic, and Endocrine Disorders

Chapter 49 Vitamin Disorders

Chapter 50 Granulomatous Diseases

Chapter 51 Disorders with Hypereosinophilia

IX

1195

1213

1229

1237

1253

1261

1293

1309

1327

1335

1345

1349

1379

1401

x

Chapter 52 Nevi

Chapter 53 Cysts and Sinuses

Chapter 54 Benign Epithelial Tumors

Chapter 55 Premalignant Epithelial Tumors

Chapter 56 Malignant Epithelial Tumors

Chapter 57 Adnexal Tumors

Chapter 58 Melanocytic Lesions

Chapter 59 Mesenchymal and Neural Tumors

Chapter 60 Pseudolymphomas

Chapter 61 Malignant Lymphomas

Chapter 62 Cutaneous Aspects of Leukemia

Chapter 63 Mastocytoses

Chapter 64 Histiocytoses

Chapter 65

Contents

Para neoplastic Markers and Syndromes

Chapter 66 Diseases of Black Skin

Chapter 67 Dermatologic Proctology

Chapter 68 Andrology

Chapter 69 Topical Therapy

1407

1423

1441

1449

1491

1511

1553

1603

1611

1681

1701

1719

Chapter 70 Systemic Therapy

Chapter 71 Physical Methods ofTherapy

Chapter 72 Operative Dermatology

Subject Index

Contents XI

1747

1781

1817

Contributing Autors

ROSELYN E. Epps, M.D. Chairperson Department of Pediatric Dermatology Children's National Medical Center 111 Michigan Avenue NW Washington, D.C. 20010, USA (Chapter 66)

JOHN A.KENNEY,JR.,M.D. Emeritus Professor and Chairman Department of Dermatology Howard University School of Medicine 2041 Georgia Avenue NW Washington, D.c. 20060, USA (Chapter 66)

Privatdozent Dr. med. RAINER ROMPEL Director Department of Dermatology Klinikum Kassel M6nchebergstraBe 41- 43 D-34125 Kassel, Germany (Chapter 72)

Prof. Dr. med. WOLF-BERNHARD SCHILL Professor and Chairman Center for Dermatology and Andrology Justus Liebig University GaffkystraBe 14 D-35385 Giessen, Germany (Chapter 68)

Privatdozent Dr. med. HANS WOLFF Department of Dermatology and Allergology Ludwig Maximilians University FrauenlobstraBe 9-11 D-80337 Munich, Germany (Chapter 68)