Abstract
A case of extramammary Paget's disease of the perianal skin with widespread metastases in a fifty-nine year old white man is presented. This represents approximately the twentyninth recorded example of this disease, and only the second in which widespread metastatic disease has been unequivocally demonstrated. Analysis of previous examples of extramammary Paget's disease reveals it to represent a primary carcinoma of the sweat glands associated with epidermal changes with cutaneous manifestations similar to those of Paget's disease of the breast. The occurrence of the epidermal involvement represents the only morphologic and clinical feature differentiating this disease from other examples of metastasizing carcinoma of the sweat glands. Morphologic evidence is presented which indicates that the primary tumor in this case was derived from apocrine glands, and that the so-called “Paget cells” represent its epidermal metastases.
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