Extramammary Paget Disease

EMPD is an intraepidermal adenocarcinoma most often limited to the epidermis, with typical cases affecting genital skin in women and men. In patients with invasive disease, prognosis is based on the degree of invasion, with tumors less than 1 mm deep having very low mortality. Careful evaluation for an underlying malignancy should be carried out to exclude life-threatening disease, but the association of many coexistent malignancies is controversial. Reported underlying adnexal adenocarcinoma may, at least in some patients, represent invasive spread of primary epidermal disease. Mohs surgery has proven effective in the treatment of EMPD, and the implementation of CK7 immunostaining shows promise in further reducing the recurrence rate. Alternative treatments, including topical treatment with imiquimod and 5-fluorouracil as well as photodynamic therapy, may be effective in select cases of EMPD.