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Triple extramammary Paget’s disease, which consists ordinarily of b ilateral axillary and genital lesions, is uncommon. Triple extramammary Paget’s diseas e involving other sites has never been reported, although solitary extramammary Paget’s disease can occur at various sites around the body, Erythematous plaqu es on the areola, axilla and genitalia of a 91- year-oldman were surgically r emoved under the clinical diagnosis of multiple extramammary Paget’s disease, Histology revealed that all three lesions consisted of intraepidermal nests of P aget cells and other isolated Paget cells scattered in the epidermis. Although a dnexal invasion was observed in the genital lesion, neither intraductal invasion nor underlying breast carcinomawas detected in the areolar lesion. Immunohistoc hemically, the Paget cells in all lesions expressed simple epithelial cytokerati ns (CK8, 18 and 19), mucin (MUC)1 and MUC5AC, but neither CK20 nor MUC2. From th e histological findings, the present case was interpreted as triple extramammary Paget’s disease rather than synchronous mammary and extramamma ry Paget’s disease. Furthermore, the mucin core protein expression pattern, wh ich was identical to that observed in extramammary Paget’s disease, supported the above interpretation.
Triple extramammary Paget’s disease, which consists ordinarily of b ilateral axillary and genital lesions, is uncommon. Triple extramammary Paget’s diseas e involving other sites has never been reported, although solitary extramammary Paget’s disease can occur at various sites around the body, Erythematous plaqu es on the areola, axilla and genitalia of a 91- year-oldman were surgically r emoved under the clinical diagnosis of multiple extramammary Paget’s disease, Histology revealed that all three of consisted of intraepidermal nests of P aget cells and other isolated Paget cells scattered in the epidermis . Although a dnexal invasion was observed in the genital lesion, neither intraductal invasion nor underlying breast carcinoma was detected in the areolar lesion. Immunohistoc hemically, the Paget cells in all lesions expressed simple epithelial cytokeratin ns (CK8, 18 and 19), mucin (MUC ) 1 and MUC5AC, but neither CK20 nor MUC2. From th e histological findings, the present case was interpreted as triple extramammary Paget’s disease rather than synchronous mammary and extramamma ry Paget’s disease. Furthermore, the mucin core protein expression pattern, wh ich was identical to that observed in extramammary Paget’s disease, supported the above interpretation.