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Extramammary Paget’s Disease

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Abstract

Extramammary Paget’s disease (EMPD) is a rare, slow-growing intraepithelial adenocarcinoma typically localized to areas of apocrine gland distribution outside the mammary glands. EMPD typically involves the vulva, perianal area, and male genitalia, with rare sites including the thighs, buttocks, axilla, eyelids, and external ear canal. The vulva remains the most frequently involved site with 65% of EMPD located in this area. Lesions appear as erythematous, brown or white, moist plaques that are well differentiated and often accompanied by subtle subjective symptoms of pruritus and pain. Its pathogenesis has been debated, but most cases are thought to arise as a primary intraepidermal neoplasm of glandular origin. A minority of disease appears to be intraepithelial spread of an underlying dermal adnexal malignancy or a regional neoplasm with contiguous epithelium. The diagnosis of EMPD is confirmed histologically by the presence of vacuolated Paget cells in the epidermis that stain for glandular cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen. Treatment options which include surgical excision, Mohs micrographic surgery, radiotherapy, photodynamic therapy, topical imiquimod 5% cream, 5-fluorouracil, and systemic chemotherapy have also been used successfully. Recurrence is common, necessitating a close follow-up.

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Authors and Affiliations

  1. Dermatology, St. Joseph’s Hospital and Medical Center, Dignity Health, Phoenix, AZ, USA

    Nathalie C. Zeitouni

  2. Tucson Hospital Medical Education Program, Tucson Medical Center, Tucson, AZ, USA

    Jose A. Cervantes

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Correspondence to Nathalie C. Zeitouni .

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  1. Department of Medicine, Division of Dermatology, Vanderbilt University Medical Center, Nashville, Tennessee, USA

    Allison Hanlon

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Zeitouni, N.C., Cervantes, J.A. (2018). Extramammary Paget’s Disease. In: Hanlon, A. (eds) A Practical Guide to Skin Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-74903-7_11

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