Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis

Rohan R Shah; Kalee Shah; Britney N Wilson; Madeline Tchack; Klaus J Busam; Andrea Moy; Mario M Leitao; Miguel Cordova; Neil M Neumann; Jan Smogorzewski; Kevin A Nguyen; Sharif Hosein; Mirabel Dafinone; Robert A Schwartz; Anthony Rossi

doi:10.1016/j.jaad.2023.07.1051

Extramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis

J Am Acad Dermatol. 2024 Sep;91(3):409-418. doi: 10.1016/j.jaad.2023.07.1051. Epub 2024 May 2.

Authors

Affiliations

¹ Department of Dermatology, Rutgers New Jersey Medical School, Newark, New Jersey.
² Department of Dermatology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, New York.
³ Department of Pediatrics, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts.
⁴ Department of Dermatology, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey.
⁵ Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York.
⁶ Department of Gynecology, Memorial Sloan Kettering Cancer Center, New York, New York.
⁷ Department of Medicine, Harbor-UCLA Medical Center, Los Angeles, California.
⁸ Department of Dermatology, West Virginia University, Morgantown, West Virginia.
⁹ Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
¹⁰ Department of Dermatology, Reno School of Medicine, University of Nevada, Reno, Nevada.
¹¹ Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York. Electronic address: [email protected].

PMID: 38704032
DOI: 10.1016/j.jaad.2023.07.1051

Abstract

Extramammary Paget disease (EMPD) is a rare skin cancer of apocrine-rich skin that mimics common inflammatory and infectious dermatoses, leading to delays in diagnosis and increased patient morbidity. Better clinical recognition of this entity, multidisciplinary patient assessment, and deeper understanding of the underlying pathophysiology are essential to improve patient care and disease outcomes. It is important to distinguish primary intraepithelial/micro-invasive EMPD from invasive EMPD or cases with adenocarcinoma arising within EMPD. This 2-part continuing medical education series provides a complete picture of EMPD. Part 1 of this continuing medical education series reviews the epidemiology, oncogenesis, clinical and histopathologic presentation, workup, and prognosis of this rare cancer.

Keywords: Extramammary Paget disease; Paget cells; immunohistochemical markers; primary EMPD; secondary EMPD; sentinel node biopsy.

Publication types

Review

MeSH terms

Diagnosis, Differential
Female
Humans
Male
Paget Disease, Extramammary* / diagnosis
Paget Disease, Extramammary* / epidemiology
Paget Disease, Extramammary* / pathology
Prognosis
Skin Neoplasms* / diagnosis
Skin Neoplasms* / epidemiology
Skin Neoplasms* / pathology