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Extramammary Paget Disease

22

 

Bradley G. Merritt and David G. Brodland

 

Abstract

EMPD is an intraepidermal adenocarcinoma most often limited to the epidermis, with typical cases affecting genital skin in women and men. In patients with invasive disease, prognosis is based on the degree of invasion, with tumors less than 1 mm deep having very low mortality. Careful evaluation for an underlying malignancy should be carried out to exclude life-threatening disease, but the association of many coexistent malignancies is controversial. Reported underlying adnexal adenocarcinoma may, at least in some patients, represent invasive spread of primary epidermal disease. Mohs surgery has proven effective in the treatment of EMPD, and the implementation of CK7 immunostaining shows promise in further reducing the recurrence rate. Alternative treatments, including topical treatment with imiquimod and 5-fluorouracil as well as photodynamic therapy, may be effective in select cases of EMPD.

Keywords

Extramammary Paget disease • Adnexal adenocarcinoma • Apocrine glands • Mohs surgery • CK7 Immunostaining

B.G. Merritt (*)

Department of Dermatology, UNC – Chapel Hill, Chapel Hill, NC, USA

e-mail: bgm2705@yahoo.com

D.G. Brodland

Departments of Dermatology and Otolaryngology, Shadyside

Medical Center, University of Pittsburgh Medical Center,

Pittsburgh, PA, USA

Summary: Introduction

EMPD is an intraepidermal adenocarcinoma usually limited to the epidermis, typically involving genital skin in women and men.

Careful evaluation for an underlying malignancy should be carried out to exclude lifethreatening disease, but the association of many coexistent malignancies is controversial.

EMPD often shows dramatic subclinical spread. Recommended margins range from 1 to 5 cm.

K. Nouri (ed.), Mohs Micrographic Surgery,

263

DOI 10.1007/978-1-4471-2152-7_22, © Springer-Verlag London Limited 2012