PATHOLOGY OF VASCULAR SKIN LESIONS - PART 1 pptx

PATHOLOGY OF VASCULAR SKIN LESIONS CLINICOPATHOLOGIC CORRELATIONS Omar P. Sangüeza, MD Luis Requena, MD HUMANA PRESS CD-ROM Included P ATHOLOGY OF V ASCULAR S KIN L ESIONS Sangueza_FM_Final 01/30/2003, 1:29 PM1 CURRENT CLINICAL PATHOLOGY IVAN DAMJANOV, MD SERIES EDITOR Pathology of Vascular Skin Lesions: Clinicopathologic Correlations, by Omar P. Sangüeza, MD, and Luis Requena, MD, 2003 Practical Immunopathology of the Skin, by Bruce R. Smoller, MD, 2002 Sangueza_FM_Final 01/30/2003, 1:29 PM2 PATHOLOGY OF VASCULAR SKIN LESIONS C LINICOPATHOLOGIC C ORRELATIONS HUMANA PRESS TOTOWA, NEW JERSEY OMAR P. S ANGÜEZA, MD Departments of Pathology and Dermatology Wake Forest University School of Medicine Winston-Salem, NC LUIS REQUENA, MD Department of Dermatology Fundación Jiménez Díaz Universidad Autonoma Madrid, Spain Sangueza_FM_Final 01/30/2003, 1:29 PM3 © 2003 Humana Press Inc. 999 Riverview Drive, Suite 208 Totowa, New Jersey 07512 www.humanapress.com All rights reserved. No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording, or otherwise without written permission from the Publisher. The content and opinions expressed in this book are the sole work of the authors and editors, who have warranted due diligence in the creation and issuance of their work. The publisher, editors, and authors are not responsible for errors or omissions or for any consequences arising from the information or opinions presented in this book and make no warranty, express or implied, with respect to its contents. Due diligence has been taken by the publishers, editors, and authors of this book to assure the accuracy of the information published and to describe generally accepted practices. The contributors herein have carefully checked to ensure that the drug selections and dosages set forth in this text are accurate and in accord with the standards accepted at the time of publication. Notwithstanding, as new research, changes in government regulations, and knowledge from clinical expe- rience relating to drug therapy and drug reactions constantly occurs, the reader is advised to check the product information provided by the manufacturer of each drug for any change in dosages or for additional warnings and contraindications. This is of utmost importance when the recommended drug herein is a new or infrequently used drug. It is the responsibility of the treating physician to determine dosages and treatment strategies for individual patients. Further it is the responsi- bility of the health care provider to ascertain the Food and Drug Administration status of each drug or device used in their clinical practice. The publisher, editors, and authors are not responsible for errors or omissions or for any consequences from the application of the information presented in this book and make no warranty, express or implied, with respect to the contents in this publication. Cover design by Patricia F. Cleary. Cover illustration: Histopathologic features in plaque lesions of Kaposi’s sarcoma: The “promontory” sign evident around preexisting capillaries. (See Fig. 9C in Chapter 9 and companion CD-ROM and discussion on pp. 223–224.) This publication is printed on acid-free paper. ∞ ANSI Z39.48-1984 (American National Standards Institute) Permanence of Paper for Printed Library Materials. Photocopy Authorization Policy: Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by Humana Press Inc., provided that the base fee of US $20.00 per copy is paid directly to the Copyright Clearance Center at 222 Rosewood Drive, Danvers, MA 01923. For those organizations that have been granted a photocopy license from the CCC, a separate system of payment has been arranged and is acceptable to Humana Press Inc. The fee code for users of the Transactional Reporting Service is: [1-58829-182-0/03 $20.00]. Printed in the United States of America. 10 9 8 7 6 5 4 3 2 1 Library of Congress Cataloging-in-Publication Data Sangüeza, Omar P. Pathology of vascular skin lesions : clinicopathologic correlations / Omar P. Sangüeza, Luis Requena. p. ; cm. (Current clinical pathology) Includes bibliographical references and index. ISBN 1-58829-182-0; 1-59259-360-7 (e-book) 1. Skin Blood vessels Tumors. 2. Skin Blood-vessels Pathophysiology. I. Requena, Luis. II. Title. III. Series. [DNLM: 1. Skin Neoplasms pathology. 2. Skin Diseases, Vascular pathology. WR 500 S226p 2003] RC280.S5 S264 2003 616.99'27707 dc21 2002027332 Sangueza_FM_Final 01/30/2003, 1:29 PM4 To Catherine, Edith, and Charles, my beloved family. Without their love, support, and patience neither this book nor any other enterprise would have been possible. To Koki, in appreciation of her love and limitless patience. Without her, nothing makes sense. Sangueza_FM_Final 01/30/2003, 1:29 PM5 Sangueza_FM_Final 01/30/2003, 1:29 PM6 Why a book on cutaneous vascular proliferations? There are several compelling reasons to justify the existence of a book on this topic. One of the most important is that cutaneous vascular proliferations are exceedingly common and affect a large number of individuals of both sexes and within a wide age range. They make up a broad spectrum of lesions with morphological and biological variations, ranging from hamartomas to highly malignant, aggressive neoplasms. Although the diagnosis of some vascular lesions is straightforward, many entities pose significant problems in diagnosis, classification, and treatment. Within the past two decades there has been an increase in the number of patients affected with Kaposi’s sarcoma, related to the epidemic of the acquired immunodeficiency syndrome (AIDS). As a consequence, a number of variants and vascular lesions that simulate Kaposi’s sarcoma, both clinically and histopathologically, have been described. In addition, other vascular entities not related to Kaposi’s sarcoma have been introduced in the literature. All of these have added confusion to an already complicated field. Since there are no recent textbooks on this subject, we felt an update was overdue. The aim of Pathology of Vascular Skin Lesions: Clinicopathologic Correlations is to provide a comprehensive and in-depth review of all vascular proliferations involving the skin and subcutaneous tissue, including recently described entities. Although our work is primarily directed to pathologists, dermatologists, and dermatopathologists, its wide scope will make it useful to pediatricians and plastic surgeons as well. Pathology of Vascular Skin Lesions: Clinicopathologic Correlations is divided into three parts. The first part covers classification and nomenclature of vascular neoplasms, an area that is still controversial. We propose a new classification with the hope that it will bring more order into a chaotic arena. We recognize that this classification may have some pitfalls and limitations, but we also believe that it is the most logical way to approach the study of vascular proliferations. In order to know what is abnormal, a student of the field should first know what is normal, which is the reason for including a chapter on normal embryology, histology, and anatomy of the skin vasculature. Another chapter is devoted to the use of special techniques for the study of vascular proliferations. In the second part, we include benign proliferations ranging from hamartomas and malformations to benign neoplasms. The final part of the book deals with malignant vascular proliferations, ranging from Kaposi’s sarcoma to angiosarcomas. It includes some new entities, too. The whole of Pathology of Vascular Skin Lesions: Clinicopathologic Correlations was conceived in terms of a clinicopathologic correlation. The clinical and morphologic vii PREFACE Sangueza_FM_Final 01/30/2003, 1:29 PM7 aspects of each entity are described in detail, including their differential diagnosis, prognosis, and therapy. Each chapter is fully illustrated with both clinical and histopathologic photographs, and we include color versions of all illustrations on the accompanying CD-ROM. Additionally, there is a complete and updated list of references for each particular section. We hope that you find this book interesting and useful. This book was sponsored in part by Pathologists Diagnostic Services, PLLC, in Winston-Salem, NC. Omar P. Sangüeza, MD Luis Requena, MD viii Preface Sangueza_FM_Final 01/30/2003, 1:29 PM8 ACKNOWLEDGMENTS Many colleagues contributed clinical pictures, histopathologic slides, or other material for this review. We are very grateful to the following clinicians and pathologists: A. Bernard Ackerman, MD (USA) Antonio Aguilar, MD (Spain) Adolfo Aliaga, MD (Spain) Isabel Febrer, MD (Spain) M. Alba Greco, MD (USA) Gerardo Jaqueti, MD (Spain) Esperanza Jordá, MD (Spain) Helmut Kerl, MD (Austria) Heinz Kutzner, MD (Germany) Pablo Lázaro, MD (Spain) Beatriz López, MD (Spain) José M. Mascaró, MD (Spain) Thomas Mentzel, MD (Germany) Special thanks to: Di Lu, MD (USA), who spent countless hours shooting micro- photographs; Rita O. Pichardo, MD (Venezuela), who helped to compile and organize all photographic and written material, and to Steven Vogel, MD, who corrected the manu- script, offered support, and provided advice. Figures 10 and 19 in Chapter 6, Figure 13 in Chapter 7, Figure 15 in Chapter 8, Figure 3 in Chapter 11, and Figure 35 in Chapter 8 have been previously published (J Am Acad Dermatol 1977;37:523-49. J Am Acad Dermatol 1997;37:887-20. J Am Acad Dermatol 1998;38:143-75). These figures are reproduced here with permission of Mosby Inc. Paula E. North, MD (USA) Celia Requena, MD (Spain) Jorge L. Sánchez, MD (Puerto Rico) Evaristo Sánchez Yus, MD (Spain) Pastor Sangüeza, MD (Bolivia) Andrés Sanz, MD (Spain) Jaime Tschen, MD (USA) Antonio Torrelo, MD (Spain) Sara O. Vargas, MD (USA) Antonio Vélez, MD (Spain) Angel Vera, MD (Spain) Michel Wassef, MD (France). ix Sangueza_FM_Final 01/30/2003, 1:29 PM9 [...]... Cytogenetic study of Kaposi’s sarcoma associated with acquired immunodeficiency syndrome Arch Pathol Lab Med 19 88 ;11 2:825–8 02/Sangüeza/ 7 -1 4/F 13 01/ 14/2003, 10 : 41 AM 14 02/Sangüeza/ 7 -1 4/F Sangüeza and Requena / Pathology of Vascular Skin Lesions 14 01/ 14/2003, 10 : 41 AM Chapter 3 / Cutaneous Vascular Proliferations 3 15 Classification of Cutaneous Vascular Proliferations The classification of vascular neoplasms... Cutaneous Vascular Hyperplasias 99 1 2 3 4 5 Angiolymphoid Hyperplasia with Eosinophilia 99 Pyogenic Granuloma 10 5 Bacillary Angiomatosis 11 2 Verruga Peruana 11 6 Intravascular Papillary Endothelial Hyperplasia (Masson’s Pseudo-Angiosarcoma) 11 9 6 Pseudo-Kaposi’s Sarcoma 12 3 7 Reactive Angioendotheliomatosis 12 8 8 Benign Neoplasms 13 3 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 9 Angioma... against KG-1a cells J Immunol 19 84 ;13 3 :15 7–65 9 Suster S, Wong TY On the discriminatory value of anti-HPCA -1 (CD-34) in the differential diagnosis of benign and malignant cutaneous vascular proliferations Am J Dermatopathol 19 94 ;16 :355–63 10 Aziza JMC, Selves J, Voigt J-J, et al Comparison of the reactivities of monoclonal QBEND10 (CD34) and BNH9 in vascular tumors Appl Immunohistochem 19 93 ;1: 51 11 Schlingemann... Skin, 8th ed Philadelphia, Lippincott-Raven, 19 97:889–932 7 LeBoit PE, Sangueza OP, Requena L Vascular neoplasms of the skin In: Farmer ER, Hood AF, eds Pathology of the Skin, 2th ed New York, McGraw-Hill 2000 :12 43–304 03/Sangüeza /1 5 -1 8/F 18 01/ 14/2003, 10 :47 AM Chapter 4 / Cutaneous Vascular Hamartomas 4 19 Cutaneous Vascular Hamartomas CONTENTS PHAKOMATOSIS PIGMENTOVASCULARIS ECCRINE ANGIOMATOUS HAMARTOMA... Pathol 19 94 ;18 :992–8 17 Weiss SW, Nickoloff BJ CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions Am J Surg Pathol 19 93 ;17 :10 39–45 02/Sangüeza/ 7 -1 4/F 12 01/ 14/2003, 10 : 41 AM Chapter 2 / Special Techniques 13 18 Burgio VL, Zupo S, Roncella S, Zocchi M, Ruco LP, Baroni CD Characterization of EN4 monoclonal antibody: a reagent with CD 31 specificity... angiomyxoma (continued) 03/Sangüeza /1 5 -1 8/F 17 01/ 14/2003, 10 :47 AM 18 Sangüeza and Requena / Pathology of Vascular Skin Lesions Table 1 (Continued) Classification of Cutaneous Vascular Anomalies 8 Disorders erroneously considered vascular neoplasms Kimura’s disease “Malignant” angioendotheliomatosis (intravascular lymphomatosis) Acral pseudolymphomatous angiokeratoma of children (APACHE) at any point... (26) A pathogenetic role of HHV-8 in KS is unequivocal The viral genome contains cellular genes that stimulate cell growth and angiogenesis; the virus is present in all clinical variants of KS, and, among all high-risk groups, seroconversion precedes the develop- 02/Sangüeza/ 7 -1 4/F 11 01/ 14/2003, 10 : 41 AM 12 Sangüeza and Requena / Pathology of Vascular Skin Lesions ment of KS The virus is detectable... Angiokeratoma in Children (APACHE) 309 Index 311 Sangueza_FM_Final 13 01/ 30/2003, 1: 29 PM Sangueza_FM_Final 14 01/ 30/2003, 1: 29 PM Chapter 1 / Vasculature of the Skin 1 1 Embryology, Anatomy, and Histology of the Vasculature of the Skin CONTENTS EMBRYOLOGIC ASPECTS ANATOMIC AND HISTOLOGIC ASPECTS OF THE DERMIS AND BLOOD VESSELS The skin is a complex organ responsible for numerous physiologic... CD 31 and CD34 in Kaposi sarcoma J Clin Pathol 19 95;48 :10 11 6 14 Kutzner H Expression of the human progenitor cell antigen CD34 (HPCA -1 ) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue J Am Acad Dermatol 19 93; 28: 613 –7 15 Van de Rijn M, Rouse R CD34: a review Appl Immunohistochem 19 94;2: 71 16 Westra WH, Gerald WL, Rosai J Solitary fibrous... Invest Dermatol 19 86;86:57–62 14 Terence RJ Structure and function of lymphatics J Invest Dermatol 19 89;93 :18 S–24S 01/ Sangüeza/ 1- 6 /F 6 01/ 14/2003, 10 :32 AM Chapter 2 / Special Techniques 2 7 Special Techniques for the Study of Vessels and Vascular Proliferations CONTENTS IMMUNOHISTOCHEMICAL STAINS MOLECULAR TECHNIQUES CYTOGENETIC STUDIES The recognition of vascular lesions is most often straightforward; . ; cm. (Current clinical pathology) Includes bibliographical references and index. ISBN 1- 5 882 9 -1 8 2-0 ; 1- 5 925 9-3 6 0-7 (e-book) 1. Skin Blood vessels Tumors. 2. Skin Blood-vessels Pathophysiology 309 Index 311 Contents xiii Sangueza_FM_Final 01/ 30/2003, 1: 29 PM13 Sangueza_FM_Final 01/ 30/2003, 1: 29 PM14 Chapter 1 / Vasculature of the Skin 1 1 1 Embryology, Anatomy, and Histology of the Vasculature. fifth gestational month. 01/ Sangüeza/ 1- 6 /F 01/ 14/2003, 10 :32 AM1 2 Sangüeza and Requena / Pathology of Vascular Skin Lesions Thereafter, there is increased production of keratohyalin granules,