Painful Purple Papules: An Interesting Case of Visceral Kaposi Sarcoma

Abstract

Introduction: AIDS-related Kaposi sarcoma (KS) is the most common tumor arising in HIV-infected persons. However, visceral involvement as the initial manifestation of KS is relatively uncommon. Case Report: A 24 year old African American male with recently diagnosed AIDS (CD4 count 50) presented with three weeks of progressive, non-radiating, cramping, peri-umbilical abdominal pain. The pain was associated with nausea, non-bloody emesis and weight loss. On physical exam, he had tenderness in his right upper quadrant and peri-umbilical region. He was also noted to have several cutaneous violaceous nodules on the nasal tip, chest wall, abdominal wall, and left lower extremity. His laboratory evaluation was notable for WBC 4.1, Hgb 8.7, platelets 458, iron 10, and ferritin 3. A contrasted computed tomography scan of the abdomen and pelvis was notable for extensive peritoneal necrotic lymphadenopathy. Esophagogastroduodenoscopy and endoscopic ultrasound revealed multiple raised isolated violaceous or hemorrhagic nodules in the oropharynx, esophagus, stomach and duodenum. Biopsies of the lesions were consistent with KS. Given his symptomatic visceral disease, he was started on chemotherapy with doxorubicin in addition to anti-retroviral therapy. Discussion: Of the four epidemiologic forms of KS, AIDS-related KS is most commonly associated with visceral disease. KS has been observed in almost all visceral sites, but the most frequent sites of non-cutaneous disease are the oral cavity, gastrointestinal tract, and respiratory system. Visceral involvement as the initial manifestation of the disease is uncommon and portends a poor prognosis. Gastrointestinal involvement can occur even in the absence of cutaneous disease; lesions may be silent or may cause abdominal pain, upper or lower gastrointestinal bleeding, nausea and vomiting, malabsorption, weight loss and/or intestinal obstruction. Gastrointestinal lesions can occur anywhere along the gastrointestinal tract, and can be easily recognized by an endoscopist as isolated or confluent hemorrhagic lesions. The diagnosis of KS should be confirmed by biopsy, although lesions tend to be submucosal, which can make histologic diagnosis difficult. Therapy is dictated by the extent of disease, as well as the presence or absence of symptoms. Severe and symptomatic disease is usually managed with anti-retroviral therapy and systemic chemotherapy.Figure 1Figure 2Figure 3