Hemophagocytic lymphohistiocytosis manifesting as a purpuric patch

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, systemic inflammatory syndrome that characteristically presents hemophagocytic histiocytes infiltrating into various organs. Common features of HLH are abrupt onset of high fever, malaise, lymphadenopathy, hepatosplenomegaly, and abnormal laboratory findings including pancytopenia, hypertriglyceridemia, hypofibrinogenemia, and high ferritin levels. Cutaneous manifestations, which occur in 6∼65% of patients, can develop at any moment in the clinical course of the syndrome. Most skin eruptions in HLH patients are generalized, transient maculopapular rashes. We describe an HLH patient who first manifested cutaneous symptoms as a large purpuric patch on her thigh. A skin biopsy found high levels of hemophagocytic histiocytes.