Extramammary Paget disease (PD) is a rare entity, accounting for only 6% of all PD. It is a neoplasm that frequently presents in areas where apocrine glands are abundant, such as the anogenital region and, less frequently, the axillae. Its prevalence is unknown, but it is more common in Caucasian and postmenopausal women, with the vulvar area being the most frequently involved location. Differential diagnosis with infectious or inflammatory diseases is difficult, and a histological study of all the suspected lesions is essential. Surgery, with total excision, remains the treatment of choice, although other therapeutic approaches such as radiotherapy, photodynamic therapy, CO2 ablative laser therapy, interferon alpha, topical 5-fluorouracil (5-FU) and topical 5% imiquimod have been used with variable success. Regardless of the adopted therapy, the disease seems to follow a chronic course with high relapsing rates, ranging from 15 to 72%.

An otherwise healthy 72-year-old postmenopausal Caucasian woman was seen in our outpatient clinic due to a 2-year history of an intensely itchy vulvar plaque. The patient reported that it all began with a small erythematous and pruritic plaque on the vulvar region that gradually enlarged over a 2-year period. She had been previously observed in another medical facility and had been treated with topical corticosteroids and cryotherapy, without clinical improvement. The dermatological observation revealed an erythematous plaque in the region of the furcula and in the perianal area, covered by thin and whitish scales, with some areas of erosion. There were no similar cutaneous lesions elsewhere, nor regional lymphadenopathy. The gynecological evaluation, including rectal examination, was otherwise unremarkable.