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 geneti...

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Veröffentlicht in:	Case Reports in Dermatology 2021-01, Vol.13 (1), p.176-183
Hauptverfasser:	Rutnumnoi, Thanachat, Leeyaphan, Charussri
Format:	Artikel
Sprache:	eng
Schlagworte:	Abdomen Antigens Biopsy Cancer Case reports Case Series Case studies Chemotherapy Colonoscopy Cytokeratin Epithelial tumors extramammary paget’s disease Familial diseases familial extramammary paget’s disease Genital diseases, Female Hypertension Lymphatic system Metastasis Mutation paget’s disease of the vulvar Pathogenesis Patients Penis Siblings Skin tumors Surgery Tumors
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Beschreibung
Zusammenfassung:	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.
ISSN:	1662-6567 1662-6567
DOI:	10.1159/000514253