P88 Case report-extramammary Paget disease

Abstract

Extramammary Paget disease is an uncommon cutaneous neoplasm, most of the times morphologically and histologically identical to mammary Paget disease of the nipple. The primary difference is the anatomic location, affecting other cutaneous sites rich in apocrine glands. The diagnosis is often delayed being often misdiagnosed as eczema. The authors describe the case of a female 75 years old patient referred to the Dermatology outpatient clinic for vulvar pruritus with 8 months evolution. Physical exam showed, occupying all the inner surface of left labia minora, erythematous, dry plaque with superficial erosion or scaling. In the medial 1/3 of the lesion was evident a raised and papillary lesion with 1 cm large. Histological exam showed epidermis infiltration by Paget cells (EMA+). Complementary evaluation with tomography, echography and colonoscopy was innocent. Parcial vulvectomy was performed and the patient is currently in the 6° month of follow-up. Dubreuith described in 1901 the first case of vulvar Paget. Although rare, it should be a differential diagnosis in patients with chronic ‘eczemas’, for earlier diagnosis. Long term follow-up is necessary do detect local recurrence of the disease or erlier diagnosis of associated neolpasm.