[Kaposi's sarcoma in young adults without evidence of HIV infection].

Besides the AIDS-related form of Kaposi sarcoma (KS) there are few well-known aggressive types of this disease. These non-epidemic invasive variants of KS have been recognized in young black males of equatorial Africa and in renal transplant recipients after iatrogenic immunosuppression. We report on two white patients without known risk factors who presented an invasive clinical form of KS with negative serology for HIV infection. Patient 1: 46 year-old white male of Italian origin without known risk factors. He consulted in September 1990 because of a nephrotic syndrome associated with membranoproliferative glomerulonephritis. On physical examination he presented a violaceous nodule in one toe of the right foot. He received prednisolone with amelioration of the renal disorder, but consulted again some months later because of fever, marked weight loss, generalized enlargement of superficial lymph nodes, autoimmune hemolytic anemia and polyclonal hypergammaglobulinemia. ELISA tests, Western-blot and HIV-antigen investigations were repeatedly negative. CD4 and CD8 counts were 450 and 365 per microlitre respectively. A lymph node biopsy demonstrated KS associated to Castleman's disease. KS was also diagnosed in the skin biopsy. The hemolytic anemia was treated with prednisolone without success and KS cutaneous lesions extended to both legs and ulcerated even after a short course of bleomycin and radiotherapy. The patient died nine months later. Patient 2: 38 year-old white male of German origin, with a history of alcoholism. He was admitted to the hospital because of septic shock associated with hepatic failure. On physical examination he presented ascites, liver and spleen enlargement, and a big subcutaneous nodule on the knee covered with normal skin.(ABSTRACT TRUNCATED AT 250 WORDS)