Vulvar Disease K793xFM.indd 1 11/29/06 9:05:56 AM K793xFM.indd 2 11/29/06 9:05:56 AM Vulvar Disease A Clinicopathological Approach Edited by Debra S. Heller UMDNJ–New Jersey Medical School Newark, New Jersey, U.S.A. Robert C. Wallach New York University Medical Center New York, New York, U.S.A. New York London K793xFM.indd 3 11/29/06 9:05:56 AM Informa Healthcare USA, Inc. 270 Madison Avenue New York, NY 10016 © 2007 by Informa Healthcare USA, Inc. Informa Healthcare is an Informa business No claim to original U.S. Government works Printed in the United States of America on acid-free paper 10 9 8 7 6 5 4 3 2 1 International Standard Book Number-10: 0-8493-3793-3 (Hardcover) International Standard Book Number-13: 978-0-8493-3793-2 (Hardcover) This book contains information obtained from authentic and highly regarded sources. Reprinted material is quoted with permission, and sources are indicated. A wide variety of references are listed. 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Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Library of Congress Cataloging-in-Publication Data Vulvar disease / edited by Debra S. Heller, Robert C. Wallach. p. ; cm. Includes bibliographical references and index. ISBN-13: 978-0-8493-3793-2 (alk. paper) ISBN-10: 0-8493-3793-3 (alk. paper) 1. Vulva--Diseases. I. Heller, Debra S. II. Wallach, Robert C. [DNLM: 1. Vulvar Diseases. WP 200 V9915 2007] RG261.V8544 2007 618.1’6--dc22 2006051475 Visit the Informa Web site at www.informa.com and the Informa Healthcare Web site at www.informahealthcare.com K793xFM.indd 4 11/29/06 9:05:56 AM In loving memory of M. Renate Dische, MD, PhD—mentor, friend, inspiration, and supporter of women—and in recognition of the pioneering study and teaching of Henry C. Falk, MD. Preface Vulvar disease is a source of patient distress, provokes multiple consultations with physicians, and is a challenging diagnostic area for gynecologists, dermatologists, and pathologists. Some gynecologists may be insecure or unfamiliar with the various vulvar problems, and their training and experience may not encompass the spectrum of disease found on the vulva, especially the dermatologic entities. Dermatologists who see vulvar disease may not be as familiar with the gynecologic vulvar disease entities. Pathologists, except for the rare subspecialists with high-volume practices, can often be challenged in this area. This book is a collaboration between a gynecologic pathologist with training and experience in obstetrics and gynecology (Debra S. Heller) and a gynecologic oncologist who has a special interest in vulvar disease (Robert C. Wallach), with input from gynecologists, dermatologists, and pathologists with interest and expertise in the field. In addition, a CD-ROM containing all the images in the book is meant to provide additional teaching and reference material. This monograph is primarily intended to be a coalescence of views from the several disciplines represented, covering clinical as well as pathologic appearances, with treatment guidelines. It is hoped that this focus of attention on vulvar disease will provide a multidisciplinary reference resource to help us all better serve women with vulvar disease. Debra S. Heller Robert C. Wallach v Contents Preface . v Contributors . ix 1. An Approach to the Evaluation of Vulvar Lesions and Complaints . 1 Bernadette Cracchiolo and Gina Anderson Anatomy . 1 Etiology . 1 Special Considerations . 7 Evaluation . 8 References . 10 2. Normal Vulva and Developmental Anomalies . ................ 11 James Scurry and Kathleen Rita Melville Anatomy . 11 Histology . 18 Embryology . 29 Developmental Anomalies . 30 References . 34 3. Noninfectious Inflammation and Systemic Diseases of the Vulva . 37 Sallie M. Neill Inflammatory Dermatoses . 37 Erosive, Ulcerative, and Bullous Dermatoses . 54 Miscellaneous . 60 References . 63 4. Vulvovaginal Infections . ....................... 67 Rachel Spieldoch and Libby Edwards Viral Infections . 67 Bacterial Infections . 77 Fungal Infections . 93 Parasitic Infections . 100 References . 104 vii viii Contents 5. Pigmented Lesions of the Vulva . ...................... 109 Hope K. Haefner, Timothy M. Johnson, Lorraine L. Rosamilia, and Douglas R. Fullen Introduction . 109 Mechanisms of Hyperpigmentation . 110 Conditions with Pigmentation . 110 Melanocytic Lesions . 111 Conclusion . 129 References . 129 6. Neoplasms and Related Lesions of the Vulva . ............... 135 Debra S. Heller Cysts . 135 Benign Tumor-Like Lesions . 138 Benign Neoplasms . 143 Preinvasive (In Situ) Neoplasms of the Vulva . 157 Invasive Neoplasms of the Vulva . 161 Benign Lesions of the Urethra . 168 Malignant Lesions of the Urethra . 176 References . 176 7. Commentary . .................................... 181 Robert C. Wallach Introduction . 181 Lichen Sclerosus et Atrophicus . 181 LSA and SCC . 182 LSA Treatment . 182 Vulvar Neoplasia . 183 Pigmented Lesions . 183 Vulvar Symptoms . 184 Genital Mutilation . 184 Genital Anatomy . 185 References . 185 Index . 189 Contributors Gina Anderson Department of Obstetrics, Gynecology, and Women’s Health, UMDNJ–New Jersey Medical School, Newark, New Jersey, U.S.A. Bernadette Cracchiolo Department of Obstetrics, Gynecology, and Women’s Health, UMDNJ–New Jersey Medical School, Newark, New Jersey, U.S.A. Libby Edwards Southeast Vulvar Clinic, Charlotte, North Carolina, U.S.A. Douglas R. Fullen Department of Pathology and Dermatology, The University of Michigan Hospitals, Ann Arbor, Michigan, U.S.A. Hope K. Haefner Department of Obstetrics and Gynecology, The University of Michigan Hospitals, Ann Arbor, Michigan, U.S.A. Debra S. Heller Departments of Pathology and Laboratory Medicine, and Obstetrics, Gynecology, and Women’s Health, UMDNJ–New Jersey Medical School, Newark, New Jersey, U.S.A. Timothy M. Johnson Departments of Dermatology, Otolaryngology, and Surgery, Melanoma Clinic, The University of Michigan Hospitals, Ann Arbor, Michigan, U.S.A. Kathleen Rita Melville Nextpath, Newcastle, Australia Sallie M. Neill St. John’s Dermatology Centre, St. Thomas’ Hospital, London, U.K. Lorraine L. Rosamilia Departments of Internal Medicine and Dermatology, The Pennsylvania State University, Milton S. Hershey Medical Center, Hershey, Pennsylvania, U.S.A. James Scurry Nextpath, Newcastle, Australia Rachel Spieldoch Scottsdale, Arizona, U.S.A. Robert C. Wallach New York University Medical Center, New York University Clinical Cancer Center, New York, New York, U.S.A. ix 1 An Approach to the Evaluation of Vulvar Lesions and Complaints Bernadette Cracchiolo and Gina Anderson Department of Obstetrics, Gynecology, and Women’s Health, UMDNJ–New Jersey Medical School, Newark, New Jersey, U.S.A. ANATOMY Many women are uncertain about their genital anatomy and may present complain- ing of vaginal itching or simply itching ‘‘down there.’’ The vulva comprises the entire external genitalia from the mons pubis anteriorly to the anus posteriorly and laterally to the genito-crural folds. Included in this area are the labia majora, labia minora, clitoris, urethral meatus, vestibule, posterior fourchette, hymen, and the Bartholin’s, Skene’s, and minor vestibular glands and the vestibular bulbs. The labia majora and mons contain adipose and fibrous tissue, covered by skin with numerous sebaceous and hair follicles. The labia minora do not contain adipose tissue, and their skin has sebaceous glands but not hair follicles. On the inner aspect of the labia minora is Hart’s line, which delineates the junction between skin and the mucous membrane. The boundaries of the vulvar vestibule are Hart’s line laterally and the hymenal ring medially, and this area contains numerous glands that secrete mucus. The vestibular bulbs are located within the bulbocavernosus muscles on either side of the vestibule and contain erectile tissue. The clitoris contains both erectile tissue and a very high density of nerve fibers. The blood supply of the vulva is from branches of the internal and external pudendal arteries, and lymphatic drainage is primarily to the external and internal iliac nodes via the groins. Branches of the pudendal nerve provide most of the motor and sensory innervation to the vulva. ETIOLOGY The evaluation of vulvar lesions can be challenging because there are a variety of poten- tial causes including infectious diseases, nonneoplastic epithelial disorders, and benign and malignant neoplasms. In addition, these conditions will frequently coexist; 1 2 Cracchiolo and Anderson