A case of cytophagic histiocytic panniculitis

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A 30-year old woman was admitted to our hospital because of fever and subcutaneous nodules. Histological examination of skin biopsy specimen disclosed diffuse panniculitis with infiltration of phagocyting histiocytes and lymphocytes. At this monent, the diagnosis of Weber-Christian disease was made, and treatment with prednisolone resulted in symptomatic relief. One month later, fever and subcutaneous nodules recurred. Labolatory examination revealed pancytopenia, coagulation abnormalities, liver dysfunction and hyperlipidemia. Despite the treatment with high dose corticosteroid, cyclophoshamide, colchicine and plasmapheresis, her condition gradually deteriorated, and finally, severe bleeding tendency such as gastrointestinal bleeding and hematuria ocurred. After 11 months, she died of panperitonitis and meningitis, which complicated after the surgical treatment of colon perforation. We considered this case as cytophagic histiocytic panniculitis rather than Weber-Christian disease. Cytophagic histiocytic panniculitis is a variant of Weber-Christian disease, originally described by Winkelmann, R. K. and Bowie, W. in 1980. Main features of this disease include fever, panniculitis, hepatosplenomegaly, pancytopenia and bleeding tendency. Pathologically, infiltration of mature histiocytes to skin, liver, spleen, bone marrow or lymph nodes with hemophagocytosis is the characteristic finding. This entity might be included in reactive histiocytosis, because of its clinical similarlity and pathological findings. Here, we report a case of cytophagic histiocytic panniculitis, and discuss the pathogenesis and treatment of this rare disease.
日本臨床免疫学会の論文

A case of cytophagic histiocytic panniculitis

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