Paget Disease of the vulva is a neoplasia characterised by a primary intraepithelial proliferation of atypical glandular-type cells that may invade the dermis, or by intraepithelial proliferation of similar cells secondary to an underlying adenocarcinoma of the Bartholin gland or rectum.

It represents 20% of the vulvar neoplasia. In contrary with Paget disease of the breast where an underlying carcinoma is almost invariably present, an underlying carcinoma is detected in only 20 to 30% of cases. Paget cells can be found in skin appendages.

The cells display a finely granular amphophilic to basophilic cytoplasm. They have a conspicuous nucleoli. Typically they are found in basal and parabasal layers of the epithelium, but sometimes more superficially. Their cytoplasm is PAS and BA positive but not always (neutral and acid polysaccharids). Some cells may even contain melanin. When Paget Disease is entirely intraepithelial, the prognosis is excellent, but if there is an underlying adenocarcinoma, the prognosis depends on the stage of the tumor and the status of the regional lymph nodes, therefore being usually poor.

83 year old woman with ulcerated lesion of the vulva. Liquid medium (ThinPrep®).