Vulvar Angiokeratoma Presenting Clinically As a Genital Wart: A Case Report and Review of Literature

Background: Angiokeratomas are superficial dermal hemangiomas and hyperkeratosis that present as small papules. Clinically, these are benign and asymptomatic but may complicate by ulceration and bleeding. In rare cases, angiokeratomas may be associated with metabolic disorders including β-galactosidase deficiency (Fabry disease). We report a case of localized angiokeratoma in a young female patient presenting clinically as genital warts. Clinical Course: A 20-year old, G0P0, African-American woman referred for consultation and excision of genital lesions for 7 years with recent size increase that were presumptively diagnosed as genital warts. The lesions were confluent clusters of white, verrucoid papules located over groin/introitus. Past medical history is significant for sexually transmitted disease (including gonorrhea and chlamydia). Histopathology: Histology revealed dilated, thin-walled, endothelial-lined vascular channels within the papillary dermis. The epidermis overlying these vessels exhibited secondary changes, including hyperkeratosis and acanthosis with elongation of rete ridges that enclosed the dermal vessels. A collarette was formed around the margins of the lesion by hyperplastic epidermis. Additionally, accompanying papillomatosis and mild, deep dermal inflammatory reaction were present. P16 immunohistochemical stain was performed and was negative. Discussion: Pathophysiology of angiokeratomas is unknown. Increased venous-pressure associated with pregnancy or varicosities have been implicated in pathogenesis. They occur in association with oral contraceptive use, and post radiation therapy. There is no known association with human papilloma virus. These are benign lesions that can most often be diagnosed clinically. They usually don’t require treatment, and when necessary, local therapy including excision and cryotherapy may be used. Of note, they can resemble a number of benign and malignant skin conditions. Chronic irritation and hyperkeratosis can give a verrucous gross appearance to these lesions. Histologically, angiosarcoma and Kaposi sarcoma are included in the differential diagnosis. These malignant tumors typically are more cellular, have a greater degree of cellular atypia and infiltrative vascular spaces with poorly defined margins. Histology is the gold standard diagnostic to differentiate amongst these entities.