Nakane S, Kawabe Y, Eguchi K, Kita A, Mizokami A, Yamasaki H, Nagataki S
The First Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki City, Japan.
Clin Rheumatol. 1997 Jun;16(4):417-21. doi: 10.1007/BF02242462.
We report a 35-year-old man, who had been diagnosed with Weber-Christian disease, presented with acute onset of high fever, malaise, jaundice and hepatosplenomegaly with subcutaneous nodules. Laboratory tests showed elevated serum ferritin and liver enzymes, especially lactate dehydrogenase (LDH), with pancytopenia and coagulation abnormalities. Peripheral blood and bone marrow examinations showed erythro-, leuko- and thrombo-phagocytic histiocytes and macrophages. The patient developed the same clinical features seven years ago. Based on diagnosis of cytophagic histiocytic panniculitis, the patient was treated with steroid pulse therapy and oral cyclosporin A. The combination therapy caused a marked improvement in the clinical condition.
