Kaposi's sarcoma and sarcoidosis coexisting in lesions of HIV-seronegative patient

A 68-year-old Omani man presented with low-grade intermittent fever for 3 months associated with loss of weight, generalized pruritus, and mild recurrent skin eruptions on his forearms for 2 years. His past history showed bilateral glaucoma, cataract, and recurrent conjunctivitis for which extraction and iridectomy were done 15 years earlier. Physical examination revealed a blind right eye, mild jaundice, pallor, hepatosplenomegaly, a few well-defined, skin-colored indurated plaques on the forearms, and no palpable lymph nodes. At this stage, laboratory investigations showed iron deficiency anemia; mild thrombocytopenia and leukopenia; polymorphs, 68%; lymphocytes 32%; serum protein 5.9 g per 100 mL; albumin 3.3 g per 100 mL; repeatedly elevated alkaline phosphatase and serum glutamic oxaloacetic transaminase (SGOT); bilirubin 3 mg per 100 mL; normal serum electrolytes, Ca, phosphate, and protein electrophoresis; and elevated serum angiotensin-I-converting enzyme concentration; and negative results for the following tests; repeated HIV serologic tests, chest roentgenogram, Mantoux skin test, repeated blood culture, Widal test, rapid plasma reagin (RPR) serology for syphilis, hepatitis B and C, and alpha-fetoprotein. On a barium meal follow-through marked hepatosplenomegaly with features of portal hypertension of the liver was noted and on an isotope scan of the spleen, disclosed liver and spleen enlargement. Multiple rounded, well-defined hypodense shadows distributed in the spleen and left lobe of the liver, with enlarged paraaortic lymph nodes were noted on an abdominal computerized tomography (CT) scan. A bone marrow biopsy showed no malignant cells, no parasites, no evidence of lymphoma, and no acid-fast bacilli (AFB) of Ziehl-Neelsen (ZN) stain. A liver biopsy showed some portal fibrosis and focal liver cells necrosis with well-formed tuberculoid granulomas. The ZN stain for AFB was negative, and no fungal or other inclusions were seen. Diseases that produce granuloma in the liver were considered, and a definite diagnosis or exclusion of tuberculosis could not be made, because the ZN stain for AFB was negative. Sarcoidosis was then raised as a posssible diagnosis and a skin biopsy showed large tuberculoid granulomas in the mid and deep dermis and a few single granulomas in the upper dermis, but there was no evidence of necrosis, and AFB were not seen. Therefore, these noncaseating granulomas were very suggestive of sarcoidosis. At this stage, although there was no definite diagnosis of tuberculosis, he was treated with antituberculous drugs for 4 months with no apparent clinical response; however, his condition deteriorated and he developed new skin lesions in the form of multiple bluish-red nodules and plaques on the feet, arms, and hands and bilateral markedly enlarged, firm and fixed inguinal lymph nodes. Biopsy from the newly erupted skin lesions showed a middermal tumor, composed of irregular vascular spaces, bundles of spindle-shaped cells with numerous mitotic figures, and extravasated erythrocytes in slit-like spaces; all were consistent with Kaposi's sarcoma (KS). Noncaseating tuberculoid granulomas were still present in moderate numbers, but lymphocytic infiltration was not a feature of these granulomas. The ZN stain for AFB remained negative and the most likely diagnosis was sarcoidosis. Furthermore, a biopsy from the right inguinal lymph node showed similar histopathologic changes, including the coexistence of both the KS and the sarcoid-like granulomas. The iliac crest biopsy also showed cancellous bone with hemorrhagic, and possibly hypoplastic, marrow with several smal scattered noncaseating sarcoid-like granulomas. Neither in the lymph node nor in iliac crest biopsies was there any evidence of lymphoma or fungal infection.