Pigmented Paget's disease of the male breast: report of a case.

An 83-year-old man with pigmented Paget's disease of the breast is reported. He had a blackish swollen right nipple with bloody discharge that lasted for 8 months. Histopathology of the lesion disclosed intraepidermal and dermal atypical cells forming small clusters and underlying intraductal carcinoma. Melanocytes were dispersed in the epidermis. Dermal melanophages were also detected. The atypical cells were positive for carcinoembryonic antigen, human-milk fat globule protein and a recently described new antigen, RCAS-1, but negative for S-100 or HMB-45. We diagnosed this case as Paget's disease of the male breast. Paget's disease of the breast is usually nonpigmented and occurs almost exclusively in women. Pigmented Paget's disease of the male breast is extremely rare, and only a few cases have been reported. Albeit rare, pigmented Paget's disease has to be included in the differential diagnosis of pigmented lesions of the nipple. Copyright 2001 S. Karger AG, Basel.