Extramammary Paget diseaseExtramammary Paget’s Disease (EMPD) (EMPD) is an intraepidermal adenocarcinoma most often limited to the epidermis, with typical cases affecting the genital skin in both 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. A careful evaluation for an underlying malignancy should be performed to exclude life-threatening disease, but the association of many coexistent malignancies is controversial. Reported underlying adnexal adenocarcinomaAdnexal adenocarcinoma may represent invasive spread of primary epidermal disease in some patients. Mohs surgeryMohs surgery has proven effective in the treatmentTreatment of EMPD, and the use of CK7 immunostainingCK7 Immunostaining reduces the recurrence rateRecurrence rates. Alternative treatmentsTreatment, including topical treatmentTreatment with imiquimodImiquimod and 5-fluorouracil (5-FU) as well as photodynamic therapyPhotodynamic therapy, may be effective in certain cases of EMPD.