Extramammary Paget's disease of the vulva recurring in a skin graft

Extramammary Paget's disease of the vulva is an uncommon vulvar neoplasm with a high rate of recurrence. A 64-year-old white female with a history of extramammary Paget's disease of the vulva, excised in 1987 by skinning vulvectomy and treated again in 1993 by skinning vulvectomy with split-thickness skin grafting, presented in 1994 with recurrent Paget's disease in the area of the skin graft. Extramammary Paget's disease of the vulva may recur in an area previously totally excised and transplanted with autologous skin. Therefore, close follow-up is necessary in all patients with this pathologic entity.