Vulvar intraepithelial neoplasia grade 3 associated with Behçet’s disease

Background Little information is available regarding the association between vulvar intraepithelial neoplasia grade 3 (VIN3) and Behçet’s disease (BD). We report here concomitant VIN3 and genital ulcers in a patient with BD. Case A 44-year-old Caucasian woman with a history of BD, which had been evolving for 6 years, presented with ulcerated and papillomatous lesions on the vulva. Biopsies revealed a multifocal VIN3 positive for high-risk human papillomavirus (HPV) 33. Multiple biopsies were performed to exclude invasive cancer and VIN3 was treated with laser vaporization. Conclusion We report clinical and anatomopathological features of a rare case of multifocal, high-risk, HPV-related VIN3. We also discuss the possible pathogenesis in the context of BD, featuring chronic ulceration and intrinsic or treatment-induced immunosuppression.