Cytophagic histiocytic
panniculitis (CHP) can be a severe variant of
Weber-Christian disease characterized by the histopathologic appearance of lobular
panniculitis infiltrated by histiocytes containing blood cell fragments and by a
clinical course with marked systemic features including multiorgan failure,
hypertriglyceridemia, and coagulopathy, which 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. In 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 five 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.