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Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview

2010; Wiley; Volume: 23; Issue: 4 Linguagem: Inglês

10.1111/j.1529-8019.2010.01339.x

1529-8019

Iris K. Aronson, Sophie M. Worobec,

Immune Cell Function and Interaction

Dermatologic TherapyVolume 23, Issue 4 p. 389-402 Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview Iris K. Aronson, Iris K. Aronson Department of Dermatology, College of Medicine, University of Illinois at Chicago, Chicago, IllinoisSearch for more papers by this authorSophie M. Worobec, Sophie M. Worobec Department of Dermatology, College of Medicine, University of Illinois at Chicago, Chicago, IllinoisSearch for more papers by this author Iris K. Aronson, Iris K. Aronson Department of Dermatology, College of Medicine, University of Illinois at Chicago, Chicago, IllinoisSearch for more papers by this authorSophie M. Worobec, Sophie M. Worobec Department of Dermatology, College of Medicine, University of Illinois at Chicago, Chicago, IllinoisSearch for more papers by this author First published: 28 July 2010 https://doi.org/10.1111/j.1529-8019.2010.01339.xCitations: 29 Iris K. Aronson, MD, Associate Professor, Department of Dermatology, College of Medicine, University of Illinois at Chicago, 1801 W. Taylor Street, Suite 3E, Chicago, IL 60612, or email: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL ABSTRACT Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis that is associated with systemic features including fevers, hepatosplenomegaly, lymphadenopathy, pancytopenia, hepatic abnormalities, hypertriglyceridemia, and coagulopathy without an elevated erythrocyte sedimentation rate. The panniculitis lesions show adipose tissue lymphocytic and histiocytic infiltration along with hemophagocytosis, which may also appear in bone marrow, spleen, lymph nodes, and liver. Patients may have a rapidly fatal disease course, a longer disease course with intermittent remissions and exacerbations for many years prior to death, or a nonfatal acute or intermittent course responsive to treatment. The cytophagocytic disorder in these patients is a hemophagocytic lymphohistiocytosis (HLH), similar to the infection-activated reaction associated with perforin mutations found in familial hemophagocytic lymphohistiocytosis. HLH is a group of autoinflammatory disorders, which include macrophage activation syndrome and infection-associated hemophagocytic syndrome, which if not treated rapidly, can be fatal. The relationship of CHP and HLH is discussed. CHP associated diseases include: subcutaneous panniculitis-like T cell lymphomas; infections, connective tissue diseases, other malignancies, and the molecular disorders that cause HLH. Treatment of CHP includes: glucocorticoids in combination with cyclosporine, combined chemotherapeutic medications and most recently, anakinra, an Interleukin-1 receptor antagonist; along with supportive care, search for underlying malignancies and treatment thereof, and control of associated infections. References 1 Winkelmann RK, Bowie EJW. Hemorrhagic diathesis associated with benign histiocytic cytophagic panniculitis and systemic histiocytosis. Archives Int Med 1980: 140 (11): 1460– 1463. 2 Crotty CP, Winkelmann RK. Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure and terminal hemorrhagic diathesis. J Am Acad Dermatol 1981: 4: 181– 194. 3 Risdall RJ, McKenna RW, Nesbit ME, et al. 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