Extramammary Paget's disease of the vulva in a young, black woman. A case report with histogenic confirmation by immunostaining.

Since the initial report, in 1901, by W. Dubreuilh of a vulvar lesion with the same histopathology as the eczematoid lesion found on the nipple and areola described seven years previously by Sir James Paget, fewer than 100 cases of this uncommon vulvar neoplasia have been reported. This disease process, with and without an accompanying adenocarcinoma, is usually found in postmenopausal Caucasian women. A 24-year-old, black woman gave a subjective history of having had a pruritic vulvar lesion for 18 months. Histologically this neoplasm proved to be extensive extramammary Paget's disease of the vulva with an accompanying adenocarcinoma involving the vulva and, unilaterally, the inguinal-femoral, pelvic and aortocaval lymphatics. Immunoperoxidase staining for carcinoembryonic antigen is valuable for differentiating this disease from similar vulvar lesions plus assessing surgical, chemotherapeutic and roentgenologic treatments for local, regional and distant disease.