Paget Disease of the Vulva: A Diagnosis not to be Forgotten

Background: Paget disease of the vulva is a rare intraepithelial adenocarcinoma.Diagnosis is based on the histopathology results.It is associated with invasive adenocarcinomas, but other synchronous neoplasms can also occur.Treatment typically consists of wide local excision or vulvectomy.Local recurrence is frequent, and it may occur several years after initial treatment. Materials & Methods:We present a case of vulvar Paget's disease treated with wide local excision.Results & Discussion: An 85-year-old patient was referred to a gynecology consultation due to complaints of persistent vulvar itching.When she was first evaluated by a gynecologist, she had mild vulvovaginal atrophy, with a whitish color along both labia majora.At this time, a course of corticosteroid therapy was attempted.When she returned about four months after the initial assessment, she presented with an eczematoid and ulcerated appearance lesion with about 7 cm on the transition between the right labia majora with labia minora.At this time, a punch biopsy was done, which confirmed Paget disease of the vulva.All additional exams came back negative.She was further submitted to wide local excision of the lesion.Currently, the patient remains under follow-up at a gynecology consultation and is stable with no recurrences of the disease. Conclusion:With this case presentation, the authors intend to emphasize the importance of considering all differential diagnosis of vulvar lesions, even those that are less frequent.Long-term follow-up is indicated in these cases because of the high risk of recurrence and the increased risk of synchronous carcinomas.