Extramammary Paget's disease of the Vulva

Vulvar Paget's disease is an extremely rare neoplasm that accounts for less than 1% of the vulvar malignancies. We present a case of a 75-year-old woman, who had an eczematoid lesion involving the labia majora and minora bilaterally, with infiltration to the clitoris. Enlarged non-fixed lymph nodes were palpable in the inguinal region bilaterally. A biopsy of the vulva showed Paget's disease. She underwent radical vulvectomy with bilateral inguinal lymph node dissection. The patient remained disease free at 6-month follow-up.