vulval Paget disease (MONDO_0002207) is 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 cytoplasm and prominent nuclei. Also known as: Paget disease of the vulva, Paget's disease of the vulva, Paget's disease of vulva, mammalian vulva Paget disease, vulva Paget's disease, vulval Paget's disease, vulvar Paget's disease.