Paget’s disease is a rare intraepithelial disorder of the vulvar skin that is seen in postmenopausal women. Unlike VIN, the intraepithelial neoplastic cells are glandular rather than squamous. The lesion primarily occurs in whites of an average age of 65 years. Grossly, it appears as a reddish, eczematoid lesion. Microscopically, this type of lesion is characterized by large pale cells that often occur in nests and infiltrate the epithelium. Once the diagnosis is made, it is important to rule out the presence of an underlying cancer. A review by Lee and colleagues reported a total of 75 cases of Paget’s disease of the vulva: 16 (22%) of the patients had underlying invasive carcinoma of the adnexal structures and 7 (9%) had adnexal carcinoma in situ.

Paget’s disease of the vulva often spreads in an occult fashion, with margins extending beyond the normal appearance of the lesion.

If there is no evidence of an underlying malignant neoplasm, a wide local excision or total vulvectomy usually is performed. If a wide local excision is performed, a slightly deeper excision is needed to remove the epidermis down to the level of the underlying fat to ensure removal of adnexal skin structures. Because this lesion extends subepithelially, a frozen section in the operating room may assist in ensuring complete removal.

Bergen and colleagues evaluated 14 patients with Paget’s disease of the vulva that was treated by vulvectomy, skinning vulvectomy with a graft, or hemivulvectomy. With a median follow-up of 50 months, all patients were free of disease; however, three patients had had locally recurrent disease.

Other modalities (topical 5-FU cream, laser) have not been used for treatment of this disease. Because both local and distant recurrence is a major risk, close follow-up is required.