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

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

Rohan R Shah  1 , Kalee Shah  2 , Britney N Wilson  3 , Madeline Tchack  4 , Klaus J Busam  5 , Andrea Moy  5 , Mario M Leitao  6 , Miguel Cordova  2 , Neil M Neumann  5 , Jan Smogorzewski  7 , Kevin A Nguyen  8 , Sharif Hosein  9 , Mirabel Dafinone  10 , Robert A Schwartz  1 , Anthony Rossi  11
Affiliations
Review

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

Rohan R Shah et al. J Am Acad Dermatol. 2024 Sep.

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.

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Conflict of interest statement

Conflict of interest Anthony Rossi: Dr Rossi has no relevant conflicts of interest related to this manuscript. He also served on advisory board, as a consultant, or given educational presentations for: Almirall; Allergan, Inc; Galderma Inc; Evolus Inc; Elekta; Biofrontera, Quantia; Merz Inc; Dynamed; Skinuvia; Perf-Action; Cutera; and LAM therapeutics. Equity in DAR companies.