Intraepithelial Extramammary Pagetʼs Disease of the Vulva: The Clinicopathological Characteristics, Management, and Outcome in a Study of 18 Female Patients

OBJECTIVE: This study aimed to analyze the clinicopathologic characteristics, management, and outcome of patients with intraepithelial extramammary Paget's disease of the vulva (EMPDV). MATERIALS AND METHODS: This retrospective study included medical records and pathological slides of 18 female patients with EMPDV between January 2001 and December 2012. Diagnosis was confirmed using conventional hematoxylin and eosin staining. RESULTS: The median age was 65 years. The distribution of lesions was from labium majus, labium minus, clitoris, and distal vaginal wall. The appearance was erythematous patches, hypopigmentation, and hyperpigmentation. The symptoms were pruritus, erosions, burning sensation, and pain. The mean duration of symptoms was 24 months. Ten patients received local excision, 3 accepted wide local excisions and successful skin grafting, and 5 underwent vulvectomies. Frozen sections showed that 3 and 15 patients had positive and negative margins, respectively. The average length of follow-up was 70 months. During a follow-up period, 3 patients were lost. One patient with positive margins and one with negative margins recurred and the overall recurrence rate was 11.1%. CONCLUSION: The diagnosis of EMPDV is often delayed but has good prognosis. A complete surgical resection is an effective treatment. Skin grafting may be a good method to promote incision healing.