Patients' Experiences With Extramammary Paget Disease: An Online Pilot Study Querying a Patient Support Group.

Publication/Presentation Date

1-1-2018

Abstract

OBJECTIVE: To illustrate the heterogeneous care delivered to patients with extramammary Paget disease (EMPD), a rare and lethal malignancy with poorly described treatment methodologies, by characterizing the clinical and pathologic characteristics of an international patient support group.

MATERIALS AND METHODS: Institutional review board approval was obtained to develop and distribute a nonvalidated survey to patients from an international, online EMPD support group. The survey was developed to capture patient clinical and pathologic details and was distributed between January 2017 and February 2017.

RESULTS: Forty-two patients completed the survey. At a mean age of 64 years, patients most commonly developed rash, pruritus, or erythema in the genital and perianal regions. Patients presented to their primary care physician, gynecologist, or dermatologist and were initially treated with topical agents for benign diagnoses. After failing conservative treatments, patients underwent biopsy by a dermatologist or gynecologist and were diagnosed with EMPD on average 21 months after the onset of symptoms. Wide local and Mohs excisions were the most frequently administered treatments with positive margins reported in 43% of patients. Fewer patients underwent noninvasive treatment with imiquimod cream and radiation. In total, 29% of patients developed regional recurrence and distant disease. There was wide variation regarding medical specialties involved, diagnostic evaluation, treatment, and clinical follow-up.

CONCLUSION: This study provides a novel view of the varied clinical and pathologic details from patients treated across varying institutions and medical specialties. This study will hopefully educate providers of the overall disease process of EMPD and encourage the development of standardized treatment recommendations.

Volume

111

First Page

214

Last Page

219

ISSN

1527-9995

Disciplines

Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods

PubMedID

28893632

Department(s)

Administration and Leadership

Document Type

Article

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