SUCCESSFUL TREATMENT OF SEVERE CYTOPHAGIC HISTIOCYTIC PANNICULITIS WITH CYCLOSPORINE-A

Cytophagic histiocytic panniculitis (CHP) can be a severe variant of W eber-Christian disease characterized by the histopathologic appearance of lobular panniculitis infiltrated by histiocytes containing blood c ell fragments and by a clinical course with marked systemic features i ncluding multiorgan failure, hypertriglyceridemia, and coagulopathy, w hich may lead to death. Therapy of CHP includes standard treatment for panniculitis, such as antimalarials, plus immunosuppressives for more severe cases. The response to treatment, however, is unpredictable. I n several recent reports, cyclosporine A has been successfully used to treat panniculitis. We report a patient and review the literature on CHP and the use of cyclosporine A as therapy. Published reports reveal that in instances of severe CHP when cyclosporine A was not given, 19 of 27 patients died (70% mortality). When severe CHP was treated with cyclosporine A, rapid remission was achieved in our patient and all f ive previously published cases (0% mortality). We believe cyclosporine A is the drug of choice in severe CHP and should be considered in all such patients. Copyright (C) 1996 by W.B. Saunders Company