Concurrence of Primary Cutaneous Extra Mammary Paget’s Disease and Squamous Cell Carcinoma in situ of Vulva: A Case Report

Background: Extramammary Paget’s disease and vulvar intraepithelial neoplasia are common lesions of vulve, but synchronous occurrence is rare in a same location. Herein we describe a concurrence of primary cutaneous extra mammary Paget’s disease and squamous cell carcinoma in situ of vulva in an Iranian women. A 59 year old woman, initially presented to Kosar teaching hospital gynecology clinic April 2017 with a single, well defined, scaly, white ulcerated mass, 2 cm in diameter on right minor labia, but other examinations were normal. She had no urinary or gastrointestinal symptoms. Incisional biopsies from the mass represented concurrence extramammary Paget’s disease and VIN3. She underwent radical vulvectomy .No evidence of disease recurrence was noted after 16 months follow up. Participant consent was obtained before patient was enrolled in this study. There are lot of diseases that involve genitalia and lead to vulvar lesions But in this rare case, we diagnosed concurrence of extramammary Paget’s disease and vulvar intraepithelial neoplasia in a same location. Additionally, the clinical presentation as a vulvar mass was found unusual. Therefore, we report the case to sensitize gynecologists and pathologists for uncommon pathologies and their manifestations in vulva. Keywords: Extramammary Paget´s disease, Intraepithelial neoplasia, Vulv