Abstract
Cytophagic histiocytic panniculitis (CHP) is a rare, usually fatal disease of childhood; it typically presents with daily high spiking fevers and severe panniculitis. Hemophagocytosis from macrophage activation during a cytokine storm can result in pancytopenia and disseminated intravascular coagulopathy. We describe a 14-year-old girl with long-standing CHP who developed severe hemophagocytic lymphohistiocytosis, which responded to treatment with a combination of high dose corticosteroids, cyclosporine, and the interleukin 1 receptor antagonist, anakinra.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Adrenal Cortex Hormones / therapeutic use
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Cyclosporine / therapeutic use
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Dose-Response Relationship, Drug
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Drug Therapy, Combination
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Female
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Humans
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Interleukin 1 Receptor Antagonist Protein / therapeutic use*
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Lymphohistiocytosis, Hemophagocytic / diagnosis
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Lymphohistiocytosis, Hemophagocytic / etiology*
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Lymphohistiocytosis, Hemophagocytic / pathology
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Panniculitis / complications*
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Panniculitis / diagnosis
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Panniculitis / drug therapy*
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Panniculitis / pathology
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Receptors, Interleukin-1 / antagonists & inhibitors*
Substances
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Adrenal Cortex Hormones
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Interleukin 1 Receptor Antagonist Protein
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Receptors, Interleukin-1
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Cyclosporine