RESUMO
Extra mammary Paget disease is rare and mostly affects perianal and vulvar zones, in patients of both sexes and from the sixth decade of life. We report a 66 years old woman with a vulvar Paget disease, subjected to a vulvectomy. Two years later, a pruriginous and erythematous plaque appeared in the perianal region. The pathological study informed an extra mammary Paget disease. The patient was subjected to a perianal excision and the correction of the surgical defect in a second intervention. After one year of follow up, there is no evidence of disease relapse.
La enfermedad de Paget de localización extramamaria (EPEM) es una patología infrecuente que afecta preferentemente a zonas perianal y vulvar en pacientes de ambos sexos y desde la sexta década de vida. Presentamos un caso clínico tratado en conjunto por los Servicios de Cirugía General, Cirugía Plástica, Ginecología y Anatomía Patológica del Hospital Universitario de Getafe.