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Extramammary Paget's Disease of the Vulva with Sclerosing Sweat Duct Carcinoma
I-Min Wu, Chung-Run Tseng, Hsien-Ching Chiu, Cheng-Hsiang Hsiao
Dermatol Sinica 19: 199-203, 2001

Extramammary Paget's Disease (EMPD) of the vulva is a rare disease manifested clinically by an itching, oozing, chronically persistent patch with burning sensation on the vulva, and histopathologically by randomly scattered single or clustered cells with hyperchromatic nuclei and abundant cytoplasm in the epidermis. Rarely, an underlying sweat gland carcinoma is found accompanying vulvar EMPD. We report a case of a 65-year-old woman with EMPD of the vulva and an underlying sclerosing sweat duct carcinoma. In this case, Paget's cells were found in the epidermis, and cords or nests of cuboidal cells with hyperchromatic nuclei and occasional ductal structures were found infiltrating the underlying sclerotic dermis. Both the Paget's cells and the infiltrating ductal cells are positive for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). The patient was treated with wide excision and split-thickness skin graft.

   
   
 
 
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