Vulvar Paget Disease

**Semantic type:** Neoplastic Process

**Definition:** An uncommon intraepithelial malignant neoplasm of eccrine or apocrine origin, arising from the vulva. It usually affects post-menopausal women. In approximately 10-20% of the cases there is an associated anorectal, or urothelial carcinoma or a skin appendage adenocarcinoma identified. It presents as a red, eczematous lesion. Microscopically, it is characterized by the presence of the typical Paget cells which are large, round cells with abundant pale cytoplasm and large nuclei with prominent nucleoli.

**Synonyms:** - Paget Disease of the Vulva - Paget Disease of the Vulva - Paget's Disease of Vulva - Paget's Disease of the Vulva - Vulva Paget's Disease - Vulvar Paget's Disease