A Case Report of Familial Extramammary Paget's Disease in Female Siblings

Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm that occurs in apocrine-bearing areas of skin. Most EMPD patients initially present with chronic pruritic eczematous lesions involving genitalia, perineum and perianal area. Familial form of EMPD is extremely rare. Several g...

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Detalles Bibliográficos
Autores principales: Rutnumnoi, Thanachat, Leeyaphan, Charussri
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2021
Materias:
Case Series
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8488423/
https://www.ncbi.nlm.nih.gov/pubmed/34703424
http://dx.doi.org/10.1159/000514253
Descripción
Sumario:Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm that occurs in apocrine-bearing areas of skin. Most EMPD patients initially present with chronic pruritic eczematous lesions involving genitalia, perineum and perianal area. Familial form of EMPD is extremely rare. Several genetic mutations have been proposed but specific modes of inheritance are still unknown. This article reports two cases of familial extramammary Paget's disease in female siblings.

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