Extramammary Paget's disease of the vulva: An annotated review of the current literature

Abstract

Extramammary Paget's disease is a rare and unusual neoplastic entity that presents mainly on apocrine gland-bearing skin. The vulva is the most common site of involvement but any area of the anogenital skin can be affected. Due to its rarity, variable clinical course and deceptive histological appearance it has a high misdiagnosis rate and both gynaecologists and dermatologists have limited experience in its management. The significance of the disease lies in its association with underlying malignancy as well as its inherent ability to invade the dermis and metastasise. The management is notoriously complicated and recurrence rates are high despite aggressive surgeries. Several alternative modalities are being explored, with results that are often variable and unpredictable. This review summarises the histopathological, clinical and therapeutic features of extramammary Paget's disease of the vulva reported in recent years.