Background: Vulvar Paget disease (VPD) is a rare skin disorder that is considered premalignant. Objective: To assess the clinical course, treatment schedules, and effect of invasion and treatment on recurrence and survival in patients with VPD. Methods: Data on women with VPD were retrieved from the medical files and pathology reports in all Dutch tertiary university medical centers. Disease-free survival and 5-year disease-specific survival were estimated by using Kaplan-Meier curves. Results: Data on 113 patients whose VPD was diagnosed between 1991 and 2016 were analyzed; 77% had noninvasive VPD. Most of the women (65%) underwent a surgical procedure. Recurrences were reported in 40%. Of the women with noninvasive VPD, 8% developed invasion. There were no disease-specific deaths reported in the women with noninvasive VPD. The 5-year disease-specific survival rate was greater than 98% in noninvasive and microinvasive VPD, but significantly worse in invasive VPD (50% [P \.0005]). Limitations: The main limitations of this study are its retrospective character and the fact that original pathology samples were not available for reassessment. Conclusions: VPD is extremely rare, and the recurrence rates are high. Most patients have noninvasive VPD, which does not affect survival and should be considered a chronic disorder with limited invasive potential. In cases of invasive disease, survival decreases significantly. ( J Am Acad Dermatol 2019;81:956-62.)

Additional Metadata
Keywords extramammary, Paget disease, recurrence, survival, vulva, vulvar neoplasms.
Persistent URL dx.doi.org/10.1016/j.jaad.2018.11.016, hdl.handle.net/1765/121276
Journal American Academy of Dermatology. Journal
Citation
van der Linden, M., Oonk, M.H, van Doorn, H.C, Bulten, J, van Dorst, E.B, Fons, G, … de Hullu, J.A. (2019). Vulvar Paget disease: A national retrospective cohort study. American Academy of Dermatology. Journal, 81(4), 956–962. doi:10.1016/j.jaad.2018.11.016