Classic Kaposi's sarcoma in two young heterosexual men

Classic Kaposi's sarcoma is primarily a skin disease of the lower extremities affecting predominantly elderly men of Mediterranean origin. We report classic Kaposi's sarcoma first presenting in the third decade in two heterosexual, HIV-negative, males of Greek origin from Albania. Ten years after onset, the disease became aggressive with unusual clinical features that included exophytic tumors, extensive lesions on the hands as well as the legs, and prominent leg edema. One of the patients also presented lesions on the face, trunk and palate, and bubonic lymphadenopathy. In both cases, CD4 counts were normal and HLA-DR5 was positive. Treatment included radiation therapy, subcutaneous interferon (alpha-2b) and combined chemotherapy (ABV). At follow up 1 and 2 years later, both patients remain in partial remission with significant improvement in clinical disease, on maintenance interferon.