[Associated Paget's disease of the vulva and of the nipple in the same patient: pathology with ultrastructural study (author's transl)].

Abstract：

:A case of Paget's disease of the vulva associated with Paget's disease of the nipple is reported. When the patient was 74-year-old, Paget's disease of the vulva was diagnosed; four years later, Paget's disease of the nipple was detected. Paget's disease of the vulva, although recurrent, is devoid of underlying infiltrating carcinoma. Eccrine sweats glands show different type of lesions consistent with pagetoid extension, in situ carcinoma and epithelial papillary proliferation. Ultrastructural study demonstrates dense granules within Paget's cells. A positive Thiery's cytochemical reaction indicates that these granules contain glycoprotein. Paget's disease of the nipple is associated with an intraductal carcinoma (cribriform type). Some feature of intralobular carcinoma is also observed. No foci of infiltration are found. Oestrogen receptors are detected by the histofluorescence technique of Sin Hang Lee. Few receptors are seen within the intraductal neoplastic cells and within the Paget's cells whereas no receptor at all, are observed within the keratinocytes. Several reports emphasized the relationship between vulvar Paget's disease and infiltrating breast carcinoma. Associated vulvar and nipple Paget's disease is rather rare and was just observed in two cases. These two observations were associated with a mammary infiltrating carcinoma whereas in our case, it remains an in situ carcinoma.