Adult onset Still's disease with persistent skin lesions complicated by secondary hemophagocytic lymphohistiocytosis

摘要

Here we describe an interesting case of a middle-aged male patient presenting with adult onset Still's disease (AOSD) complicated by secondary hemophagocytic lymphohistiocytosis (HLH) and generalized dermal and mucosal hyper-pigmentation. AOSD is a rare, inflammatory disease of unknown etiology, affecting primarily young adults, and characterized by high spiking fevers, arthritis and an evanescent, macular, non-pruritic, salmon-colored rash, distributed on the trunk and the extremities. Organomegaly and lymphadenopathy are common associations. Laboratory tests reveal neutrophilic leukocytosis, negative rheumatoid factor (RF) and antinuclear antibodies (ANA), as well as high serum ferritin levels and low serum glycosylated ferritin levels.[1] A small number of AOSD cases complicated by secondary hemophagocytic lymphohistiocytosis (HLH) have been described.[2-4]