Extramammary Paget's disease: a report of three cases and review of the literature.

Extramammary Paget's disease (FMPD) is a rare skin disease with a tendency to recur even in the face of adequate excision. It was first suggested by Weiner in 1937 to be a carcinoma of the apocrine sweat gland with intraepithelial excision. Thus, most cases of EMPD occur in the perianal, perigenital and axilla regions where sweat glands abound, with scrotal EMPD being the commonest. EMPD can occur in both benign or malignant form and is often confused with chronic dermatitis. Diagnosis can only be confirmed by an excision biopsy. The long-term survival in patients without an underlying carcinoma is good. However, EMPD associated with an underlying adnexal carcinoma tends to be more aggressive and the prognosis is poor in such cases. EMPD is also associated with other malignancies such as prostatic, rectal, cervical, breast, bladder and skin carcinomas. Incidences of up to 40% have been reported. Thus, patients with EMPD should be thoroughly screened for associated primary growths. The treatment of EMPD is essentially by surgical excision with clear margins. The options available for a clear margin include intraoperative frozen section,Moh's surgery and paraffin section with delayed re-excision. In this report, we present three cases of EMPD with markedly different outcomes and a review of the literature.