title: Paget disease of the vulva.
abstract: 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.
authors.affiliation: Department of Obstetrics & Gynaecology, Radboud university medical center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. Electronic address: Michelle.vanderLinden@radboudumc.nl.
authors.forename: M
authors.initials: M
authors.lastname: van der Linden
authors.affiliation: Department of Dermatology, Radboud university medical center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
authors.forename: K A P
authors.initials: KA
authors.lastname: Meeuwis
authors.affiliation: Department of Pathology, Radboud university medical center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Bulten
authors.affiliation: Department of Pathology, Leiden university medical center, P.O. Box 9600, 2300 RC Leiden, The Netherlands.
authors.forename: T
authors.initials: T
authors.lastname: Bosse
authors.affiliation: Department of Gynaecology, Leiden university medical center, P.O. Box 9600, 2300 RC Leiden, The Netherlands.
authors.forename: M I E
authors.initials: MI
authors.lastname: van Poelgeest
authors.affiliation: Department of Obstetrics & Gynaecology, Radboud university medical center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
authors.forename: J A
authors.initials: JA
authors.lastname: de Hullu
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D010145:Paget Disease, Extramammary / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D011379:Prognosis; D059016:Tumor Microenvironment; D014846:Vulvar Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology
keywords: Anogenital; Extramammary Paget disease; Imiquimod; Paget disease; Perineal; Surgery; Vulvar Paget disease; Vulvar carcinoma; Vulvar neoplasm