Extramammary Paget Disease

Bradley G Merritt; Catherine A Degesys; David G Brodland

doi:10.1016/j.det.2019.02.002

Extramammary Paget Disease

Dermatol Clin. 2019 Jul;37(3):261-267. doi: 10.1016/j.det.2019.02.002. Epub 2019 Apr 19.

Authors

Bradley G Merritt¹, Catherine A Degesys², David G Brodland³

Affiliations

¹ Department of Dermatology, University of North Carolina-Chapel Hill, 410 Market Street, Suite 400, Chapel Hill, NC 27516, USA.
² Department of Dermatology, University of North Carolina-Chapel Hill, 410 Market Street, Suite 400, Chapel Hill, NC 27516, USA. Electronic address: ABBIE.DEGESYS@GMAIL.COM.
³ Zitelli and Brodland PC, 5200 Centre Avenue, Suite 303, Pittsburgh, PA 15232, USA; Department of Dermatology, The University of Pittsburgh Medical Center, Pittsburgh, PA, USA; Department of Otolaryngology, The University of Pittsburgh Medical Center, Pittsburgh, PA, USA; Department of Plastic Surgery, The University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

PMID: 31084720
DOI: 10.1016/j.det.2019.02.002

Abstract

Extramammary Paget disease is an intraepidermal adenocarcinoma, most often limited to the epidermis, with typical cases affecting genital skin. When limited to the epidermis, primary extramammary Paget disease is not life-threatening, but invasive disease may portend a poor prognosis. Surgical excision remains the mainstay of treatment of extramammary Paget disease, and Mohs micrographic surgery is the surgical treatment of choice. Alternative treatments include topical 5-fluorouracil and imiquimod, photodynamic therapy, laser vaporization, chemotherapy, and radiation therapy but data are limited. Implementation of cytokeratin 7 immunostain has increased the ability to detect extramammary Paget disease on frozen section.

Keywords: CK7 staining; Extramammary paget disease; Mohs micrographic surgery.

Publication types

Review

MeSH terms

Antineoplastic Agents / therapeutic use
Humans
Mohs Surgery*
Neoplasms, Multiple Primary / diagnosis*
Paget Disease, Extramammary / diagnosis*
Paget Disease, Extramammary / therapy*
Prognosis
Skin Neoplasms / diagnosis*
Skin Neoplasms / therapy*

Substances

Antineoplastic Agents