Extramammary Paget’s Disease
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Extramammary Paget’s disease (EMPD) is a rare form of intraepithelial skin adenocarcinoma affecting most commonly the vulva. Its incidence varies between less than?1% and 2% of vulvar malignancies, with a higher incidence in postmenopausal white females. It may also affect areas rich in apocrine glands such as the groin, thigh, buttocks, perianal region, axilla, external ear canal, eyelids, penis and scrotum. There are two types of vulvar Paget’s disease, intraepithelial adenocarcinoma arising from the vulva and perineum, and pagetoid intraepithelial spread of primary carcinoma from an adjacent area. It presents as a slowly expanding asymmetrical white and red peeling plaque on the vulva associated with pruritus, pain, drainage and bleeding. This disease often spreads in an occult fashion with margins extending beyond the apparent lesion; therefore, the interventional treatment can be challenging, leading to positive surgical margins and frequent recurrences. This report describes the case of a female with non-invasive Paget’s disease of the vulva with invasive disease to urethra and lymph nodes that recurred 33?years after her initial diagnosis and management.

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