Iasian Pacific Journal of Allergy and Immunology

Weber-Christian disease (WCD) is an inflammatory disease of subcutaneous fat first described in 1892. Panush et al. I analysed the clinical and laboratory features of WCD, including chronic relapsing fever with subcutaneous non-sup­ purative nodules predominantly on lower extremities, arthralgia or ar­ thritis and myalgia. The laboratory features were elevated erythrocyte sedimentation rate, anemia, leuko­ penia and hypocomplementemia. Subsequent reports by Winklemann and Bowie in 1980, and by Crotty and Winklemann in 1981 found that individual cases of so-called Weber-Christian disease with bleeding diathesis, represented a new and unique syndrome called "cytophagic histiocytic panniculi­ tis" (CHP). CHP was characterized by a clinical course of fever, recur­ rent nodules of panniculitis, mu­ cous membrane ulcerations, cyto­ penia, serosal effusion, reticuloen­ dotheliomegaly progressing to liver dysfunction and terminal hemor­ rhagic diathesis . Pathological feaSUMMARY This is a report of a case, 7 ~ year-old-boy having chronic febrile and recurrent crops of painful SUbcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histio­ cytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a succes.ful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.