A 25-year-old man was diagnosed with HIV infection and a CD4 count of 21/μl. He presented with chills, fever up to 39.5°C and dry cough since 1 week. Antibiotic treatment for suspected pneumonia resulted in some clinical improvement. He had a blackish spot on his right forearm, which slowly evolved into a firm nodule (panel A), and a small purple spot on the hard palate. Both were interpreted as Kaposi's sarcoma (KS). Endoscopy revealed no gastrointestinal or pulmonary lesions. Human herpes virus 8 (HHV8) IgG EIA was negative both initially and after starting highly active antiretroviral therapy (HAART). Despite an increase of CD4 to 207/μl, the oral nodule evolved into a prominent tumor of 2.5x2.5 cm (panel B). Biopsy confirmed KS, showing irregular vascular spaces, prominent endothelium, extravasated erythrocytes, and atypical spindle cells positive for CD31, CD34 and HHV8 (panel C). Several weeks later, the patient developed increasing dyspnea; a CT scan revealed perihilar, radially configured pulmonary infiltrates predominantly of the left lower lobe (panel D), consistent with pulmonary KS.
HIV-associated KS affects the skin and/or mucous membranes and is strictly associated with HHV8 coinfection. However, HHV8 serology may be unreliable especially in the setting of severe immunodeficiency. Usually, immune reconstitution with HAART leads to resolution of KS, but in some cases chemotherapy may be necessary.
Treatment with liposomal doxorubicin led to rapid improvement of his dyspnea, weight gain of 3 kg within 6 weeks, and complete flattening of the lesion on the palate within 9 weeks.