PERIANAL PAGETS-DISEASE - REPORT OF A CAS E AND REVIEW OF THE LITERATURE

A Caucasian woman with an unremarkable medical history presented with typical pruritis ani with perianal erythema. Antifungal agents were in effective, and a biopsy done after ten months established the diagnosi s of Paget's disease. Extensive investigations (rectosigmoidoscopy, ba rium enema, computed tomography of the pelvis) failed to detect any ev idence of carcinoma. The anal lesion was treated by wide margin laser application. Perianal Paget's disease is 1.5 to 2 times more common in men than in women and occurs at a mean age of 64.7 years. In 60 % of cases a carcinoma is found, with anorectal carcinoma being two to thre e times mon common than sweat gland duct carcinoma. Only 207 cases of perianal Paget's disease have been reported, over a period of 104 year s (1893-1997). Only four patients were aged 80 to 85 years. The main d ifferential diagnoses are Bowen's disease and in situ pagetoid melanom a. The immunohistochemical characteristics of the Paget's cell may be useful for predicting simultaneous or metachronous anorectal carcinoma . The interval between the occurrence of the two lesions can be as lon g as 15 years. The management of early perianal Paget's lesions (witho ut anorectal carcinoma or sweat gland duct carcinoma) is based on exci sion with wide margins in all directions to reduce the risk of local r ecurrence. The boundaries of the lesion should be identified by microg raphy before the procedure. The need for skin grafting after the excis ion is not agreed on. The management of more advanced perianal Paget's lesions is similar to that of classic anorectal carcinoma. After trea tment, patients should be closely monitored for local recurrences and for anorectal carcinoma.