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Extramammary Paget disease.
Dermatol Online J 2019; 25(4)DO

Abstract

In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. EMPD presents as an erythematous plaque on apocrine gland bearing areas (i.e. vulva, perineum, perianal region, scrotum, and penis) in older men and women. It can be a focal, multifocal, or an ectopic process. Immunohistochemical staining allows for differentiation between primary and secondary EMPD in addition to the many other disease entities that clinically resemble this malignancy. When diagnosing a patient with EMPD, a full history and physical should be performed given the possibility of an underlying malignancy. Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease.

Authors+Show Affiliations

No affiliation info availableNo affiliation info availableNo affiliation info availableVeterans Affairs Medical Center Brooklyn, Brooklyn, New York SUNY Downstate, Department of Dermatology, Brooklyn, New York. amorkh@gmail.com.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31046904

Citation

St Claire, Kayla, et al. "Extramammary Paget Disease." Dermatology Online Journal, vol. 25, no. 4, 2019.
St Claire K, Hoover A, Ashack K, et al. Extramammary Paget disease. Dermatol Online J. 2019;25(4).
St Claire, K., Hoover, A., Ashack, K., & Khachemoune, A. (2019). Extramammary Paget disease. Dermatology Online Journal, 25(4).
St Claire K, et al. Extramammary Paget Disease. Dermatol Online J. 2019 Apr 15;25(4) PubMed PMID: 31046904.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extramammary Paget disease. AU - St Claire,Kayla, AU - Hoover,Alena, AU - Ashack,Kurt, AU - Khachemoune,Amor, Y1 - 2019/04/15/ PY - 2019/04/29/received PY - 2019/04/29/accepted PY - 2019/5/4/entrez JF - Dermatology online journal JO - Dermatol. Online J. VL - 25 IS - 4 N2 - In 1874, Sir James Paget first described Paget disease of the nipple, also known as mammary Paget disease. In 1889, extramammary Paget disease (EMPD) of the scrotum and penis was identified. Although mammary and extramammary Paget disease are both characterized by epidermal Paget cells and share a similar clinical presentation, their uniqueness lies in anatomical location and histogenesis. EMPD presents as an erythematous plaque on apocrine gland bearing areas (i.e. vulva, perineum, perianal region, scrotum, and penis) in older men and women. It can be a focal, multifocal, or an ectopic process. Immunohistochemical staining allows for differentiation between primary and secondary EMPD in addition to the many other disease entities that clinically resemble this malignancy. When diagnosing a patient with EMPD, a full history and physical should be performed given the possibility of an underlying malignancy. Surgical excision currently is first line therapy and the prognosis is often favorable. Recent advances within the field have examined the expression of chemokine receptors within tumors, which may be applicable in determining prognosis. This review addresses the history, epidemiology, pathogenesis, clinical presentation, histopathology, differential diagnosis, diagnosis, management, and new observations with respect to extramammary Paget disease. SN - 1087-2108 UR - https://www.unboundmedicine.com/medline/citation/31046904/Extramammary_Paget_disease L2 - http://escholarship.org/uc/item/7qg8g292 DB - PRIME DP - Unbound Medicine ER -