Paget disease of the vulva

•Vulvar Paget's disease is a rare skin disorder.•It is associated with underlying urogenital, intestinal or vulvar malignancies.•Concomitant carcinomas are very rare.•Invasive disease has worse prognosis than non-invasive disease.•Topical imiquimod cream may be an effective and safe treatment alternative.

In this review, we provide an overview of the clinical aspects, histopathology, molecular genetics, and treatment options for Vulvar Paget's Disease (VPD), a rare skin disease, most commonly found in postmenopausal Caucasian women. The underlying cause of VPD remains not well understood. VPD is rarely associated with an underlying urogenital, gastrointestinal or vulvar carcinoma. In approximately 25% of the cases, VPD is invasive; in these cases, the prognosis is worse than in non-invasive cases. Recurrence rates in invasive VPD are high: 33% in cases with clear margins, and even higher when surgical margins are not clear, regardless of invasion. Historically, surgical excision has been the treatment of choice. Recent studies show that imiquimod cream may be an effective and safe alternative.