Abstract
Quadruple extramammary Paget's disease (EMPD) is very rare. Herein we report a case of quadruple EMPD in a Japanese male. A 68-year-old Japanese male presented with a 4-year history of erythema of the umbilical lesion. After a biopsy, he was suspected as having EMPD, and was referred our hospital. Physical examination revealed erythematous lesions on his axillae and genital area, as well as on the umbilicus. Biopsy specimens from the 4 lesions exhibited tumor cells with large pleomorphic nuclei and abundant cytoplasm, which had proliferated in the intra-epidermis. Enhanced computed tomography did not reveal any signs of lymph node or visceral metastasis. Under general anesthesia, the lesions were resected with a 1 cm-margin. Histological examination revealed tumor cells with large pleomorphic nuclei and abundant cytoplasm, and were positive for PAS staining. These cells had proliferated in focal, adenomatous, or were solitary in the intra-epidermis. Immunohistological analysis showed the tumor cells to be positive for CK7 and negative for CK20. From these observations, a confirmed diagnosis of quadruple EMPD was made.[Skin Cancer (Japan) 2013 ; 28 : 283-286]
