胸部陰影で発症し, cyclosporin Aが奏効した cytophagic histiocytic panniculitis の1例

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A 28-year-old woman admitted at the Okayama University Hospital demonstrated pulmonary infiltrates, splenomegaly and pancytopenia. She received anti-tuberculous therapy for 5 months; however, the pulmonary X-ray shadows did not improve. At that time a transbronchial lung biopsy (TBLB) and bronchoalveolar lavage (BAL) were performed. TBLB showed organizing pneumonia, and lymphocytosis and decreased CD 4/CD 8 ratio was seen in the BAL fluid. Treatment with a corticosteroid was effective on the pulmonary shadows and pancytopenia. Fifteen months later, the patient was re-admitted because of high fever and hepatosplenomegaly. A hemogram revealed anemia and leukopenia, and blood chemistry studies showed liver dysfunction and coagulation abnormalities. She also had tender subcutaneous nodules. A skin showed inflammatory cell infiltration into the fat lobules, and she was diagnosed cytophagic histiocytic panniculitis (CHP). Increasing the dose of prednisolone did not influence her clinical course. We initiated treatment with ciclosporin A at a daily dosage of 300 mg, and dramatic improvement of her clinical symptoms occurred.These results suggest that T lymphocytes as well as macrophages play an important role in CHP, because ciclosporin A acts primarily on T lymphocytes and the BAL fluid revealed T lymphocytosis.