The aim of this study is to present a very rare dermatologic condition of the vulva. Angiokeratoma is a benign dilation of ectatic thin-walled blood vessels and congested capillaries in the superficial dermal layer of skin. It occurs predominantly in men and extremely rare in women. Angiokeratoma presents as single or multiple papular lesions on the vulva with smooth or verrucous surface. These lesions are easily confused with infectious disease, inflammatory lesions, and epithelial tumors. In this report, we presented a 42 year old unmarried woman who was admitted to our clinic with complaints of two papuler lesions of the right labium majus with vaginal discharge, vulvar pruritis, and vulvar edema. She had been previously treated with different local or systemic antiinfectious agents and her screening tests for sexually transmitted disease were negative. The genital examination showed a very thinned, hyperemic and edematous vulvar skin with two lesions measured 0,5-1 cm in diameter on the right labium majus. The surface of the lesions was partly uneven and partly veined. A local excision was performed. Histological examination showed hyperkeratosis, papillomatosis and acanthosis with dilated vascular areas in the dermis and the specimen revealed angiokeratoma. In conclusion, we should be aware of this clinical entity to make the differential diagnosis of a lesion on the vulva.
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