A Disease, a Syndrome, or Both?

Abstract

Journal of Gynecologic SurgeryVol. 39, No. 2 EditorialFree AccessA Disease, a Syndrome, or Both?Mitchel S. HoffmanMitchel S. Hoffman—Mitchel S. Hoffman, MD, Editor-in-Chief Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine, Tampa, Florida, USA.MCC GYN Program, Moffitt Cancer Center, Tampa, Florida, USA.Search for more papers by this authorPublished Online:12 Apr 2023https://doi.org/10.1089/gyn.2023.0025AboutSectionsPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail As a gynecologist and oncologist, I have long been interested in Paget's disease of the vulva, so the article on this condition (pp. 66–69) was particularly interesting to me.The condition, termed extramammary Paget's disease (EMPD), falls under a deceptively simple heading for what is a spectrum of potential abnormalities that characterize the syndrome. The study in this issue by Dr. Sánchez-Prieto and associates touches on several of the many interesting aspects of EMPD.Sir James Paget, 1st baronet, FRS HFRSE, a prominent professor of surgery and anatomy at St. Bartholomew's Hospital, in London, United Kingdom, in the nineteenth century, described both the bone and breast diseases that bear his name. EMPD was first described toward the end of the nineteenth century.While the disease is rare, the gynecologic surgeon will occasionally encounter EMPD—especially as the population continues to age. This disease is not only difficult to recognize but has challenging complexity.The most-distinguishing aspect of EMPD is its symptomatic skin disease that most commonly involves the external genitalia of postmenopausal Caucasian females. However, there are many manifestations of EMPD beyond the skin. Some of the other clinical characteristics of EMPD include its potential to become invasive, the phenomenon of “heralding” an underlying carcinoma (apocrine gland, urothelial, rectal), the association with cancers at remote sites (such as the breast), and the marked incongruity of clinical and microscopic extents of the skin disease—the clinical significance of which is unclear.There are a variety of therapeutic options for treating EMPD, some of which are under investigation. Wide local excision continues to be the treatment of choice for the primary skin disease. However, the importance of achieving negative margins and the methods of accomplishing this goal are still being investigated. Some recent therapeutic options include Mohs surgery and the use of fluorescein dye mapping. Management of recurrent skin disease is highly individualized and such disease may be symptomatic only in elderly women.The journal's editorial staff and editorial board are working hard to provide a combination of the best educational and research content for you, our readers. Please let us know how we are doing and feel free to provide suggestions for improvement in content.FiguresReferencesRelatedDetails Volume 39Issue 2Apr 2023 InformationCopyright 2023, Mary Ann Liebert, Inc., publishersTo cite this article:Mitchel S. Hoffman.A Disease, a Syndrome, or Both?.Journal of Gynecologic Surgery.Apr 2023.59-59.http://doi.org/10.1089/gyn.2023.0025Published in Volume: 39 Issue 2: April 12, 2023PDF download