CYTOPHAGIC HISTIOCYTIC PANNICULITIS OCCURRING IN A 4-MONTH-OLD INFANTWITH CHARACTERISTIC HYPERLIPIDEMIA

We report a 4-month-old patient with nodular erythematous skin lesions , fever, liver dysfunction, hyperlipidemia nearly similar to that in f amilial hemophagocytic lymphohistiocytosis, and a fatal outcome with t he development of pancytopenia, hepatic failure, and coagulation defec ts. Histopathology of the skin revealed accumulation of benign-looking histiocytes with hemophagocytosis and slightly atypical lymphocytes i n the subcutis. Autopsy revealed the presence of cytophagic histiocyte s in the splanchnic organs. The liver showed fatty degeneration and fo cal hepatocellular necrosis. The symptoms were most consistent with cy tophagic histiocytic panniculitis which is unusual in a pediatric age group.