Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A

B. E. Ostrov, B. H. Athreya, A. H. Eichenfield, D. P. Goldsmith

Research output: Contribution to journalArticle

38 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)404-413
Number of pages10
JournalSeminars in Arthritis and Rheumatism
Volume25
Issue number6
DOIs
StatePublished - Jan 1 1996

All Science Journal Classification (ASJC) codes

  • Rheumatology
  • Anesthesiology and Pain Medicine

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