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Management of Paget Disease of the Vulva

May 2010 – by P. DiSaia, MD

Extramammary Paget disease (EMPD) of the vulva is a rare malignant neoplasm of the vulva skin presenting mostly commonly with a compliant of pruritus. Unfortunately, diagnosis is often delayed as both patients and physicians dismiss the itch as secondary to local irritation from garments or benign vulvitis. Inspection of the vulva reveals a redness of the skin with an occasional violaceous hue. At still other times the red skin is partially covered with a thin white coating like "cupcake frosting".

Diagnosis is made by biopsy with subsequent review by a Pathologist familiar with vulva pathology and identification of Paget cells. Treatment is wide local excision. Since invasion to structures below the dermis is rare, inguinal lymphadenectomy is not necessary unless the final pathology reports invasion.

Many clinicians perform frozen section margin analysis at the time of surgery and extend the wide resection until margins are declared negative. Unfortunately, recurrence is common even when margins are clear. The disease process appears to have the ability to skip over apparent normal skin and can recur at sites distant from the surgical site. For this reason, the patients must be monitored closely post operatively and suspicious areas biopsied. In many patients multiple local excisions are performed over a period of 10-20 years.

Older literature reports adenocarcinomas of the rectum, colon and uterus associated with Paget Disease of vulva. However, more recent reports have found a very low incidence of such simultaneous lesions.