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Deficiency of interleukin-1 receptor antagonist responsive to anakinra.

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.
AuthorsCharlotte Schnellbacher, Giovanna Ciocca, Roxanna Menendez, Ivona Aksentijevich, Raphaela Goldbach-Mansky, Ana M Duarte, Rafael Rivas-Chacon
JournalPediatric dermatology (Pediatr Dermatol) 2013 Nov-Dec Vol. 30 Issue 6 Pg. 758-60 ISSN: 1525-1470 [Electronic] United States
PMID22471702 (Publication Type: Case Reports, Journal Article)
Copyright© 2013 Wiley Periodicals, Inc.
Chemical References
  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein
Topics
  • Antirheumatic Agents (therapeutic use)
  • Female
  • Hereditary Autoinflammatory Diseases (complications, drug therapy)
  • Humans
  • Infant
  • Interleukin 1 Receptor Antagonist Protein (therapeutic use)
  • Osteomyelitis (drug therapy, etiology)
  • Periostitis (drug therapy, etiology)
  • Skin Diseases, Vesiculobullous (drug therapy, etiology)

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