Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique.

The perianal skin is a common area for extra-mammary Paget's disease development. The unique clinical, histopathological, and immunohistochemical features which this medical phenomenon demonstrates, along with its rarity and frequent association with synchronous or metachronous carcinomas, present us with a treatment challenge. In order to organize the surgical treatment, it is important to determine whether the disease is localized exclusively to the perianal skin or associated with metastasis or anorectal carcinomas.