Abstract
We describe a 3-month-old infant who presented to our institution with interleukin (IL)-1 receptor antagonist deficiency (DIRA), which consists of neutrophilic pustular dermatosis, periostitis, aseptic multifocal osteomyelitis, and persistently high acute-phase reactants. Skin findings promptly improved upon initiation of treatment with anakinra (recombinant human IL-1 receptor antagonist), and the bony lesions and systemic inflammation resolved with continued therapy.
© 2013 Wiley Periodicals, Inc.
MeSH terms
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Antirheumatic Agents / therapeutic use
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Female
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Hereditary Autoinflammatory Diseases / complications
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Hereditary Autoinflammatory Diseases / drug therapy*
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Humans
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Infant
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Interleukin 1 Receptor Antagonist Protein / therapeutic use*
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Osteomyelitis / drug therapy*
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Osteomyelitis / etiology
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Periostitis / drug therapy*
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Periostitis / etiology
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Skin Diseases, Vesiculobullous / drug therapy*
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Skin Diseases, Vesiculobullous / etiology
Substances
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Antirheumatic Agents
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Interleukin 1 Receptor Antagonist Protein
Supplementary concepts
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Deficiency of interleukin-1 receptor antagonist