Background: Extramammary Paget's disease (EMPD) of the vulva is a rare clinical entity that often affects Caucasian postmenopausal women. The disease presents with vulval itching and soreness. Wide local excision, topical application of chemotherapeutic agents, and radiotherapy have been used to treat this rare condition. Case: A 65-year-old, para 3, postmenopausal woman presented with vulval itching and burning, which topical dermatologic creams failed to resolve. Local and pelvic examinations did not show any abnormality but a biopsy showed that she had Paget cells along the basal part of the squamous epithelium of the skin. The affected area was excised and the removed specimen was examined histologically, showing Pagetoid changes. This patient was treated with imiquimod cream for 6 months. Results: At a 6-month follow-up, this patient was symptom-free and had no clinical evidence of a recurrence. Conclusions: EMPD has a propensity for recurrence and, rarely, may signal an underlying adenocarcinoma. The diagnosis requires a high degree of clinical suspicion and histologic confirmation. A variety of approaches exist for treatment and prevention of potential recurrences. (J GYNECOL SURG 29:297)
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