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E
xtramammary Paget disease (EMPD) is a rare and multifocal, and subclinical lesions making the initial di-
lethal intraepithelial malignancy with poorly de- agnosis challenging. Lesions may also become invasive, pre-
scribed treatment methodologies and outcomes. In- cluding definitive treatment. EMPD has also been associated
cidence has been reported as 0.12 per 100,000 people, with with secondary internal malignancies, such as rectal, bladder,
an overall survival of 60% at 120 months post diagnosis.1,2 prostate, and endocervical cancers.3
EMPD develops most frequently in the genital, perianal, Because of the low incidence of the disease, current
and axillary regions, and commonly presents with symp- studies evaluating EMPD are based on single-institution
toms of erythematous or pruritic rash or plaque (Fig. 1). case reports and small cohort series that have not com-
Patients often initially undergo rounds of ineffective treat- prehensively assessed the clinical journey from initial disease
ments for a benign diagnosis before a correct diagnosis of presentation to diagnosis to treatment and through follow-
EMPD is obtained. EMPD may present as poorly defined, up. The impetus for our current study is the increased
number of patients with EMPD that we have treated over
the past 4 years. These patients were referred having re-
Financial Disclosure: The authors declare that they have no relevant financial ceived a variety of care by outside providers that prompted
interests. us to query patients regarding their own experiences. We
From the Department of Urology, University of Washington Medical Center, Seattle,
WA; and the Division of Dermatology, University of Washington Medical Center, Seattle, developed an online survey to illustrate the heteroge-
WA neous care delivered to patients with EMPD by charac-
Address correspondence to: Paul H. Chung, M.D., Department of Urology, Harborview terizing the clinical and pathologic characteristics of an
Medical Center, University of Washington Medical Center, 325 9th Ave., Seattle, WA
98104. Email: phchung@uw.edu international, online patient support group treated across
Submitted: June 1, 2017, accepted (with revisions): August 30, 2017 different institutions.
214 © 2017 Elsevier Inc. https://doi.org/10.1016/j.urology.2017.08.045
All rights reserved. 0090-4295
Figure 1. Representative extramammary Paget disease (EMPD) lesions that are confined to the scrotum (A, B) and locally
advanced (C). (Color version available online.)