Deficiency of interleukin-1 receptor antagonist responsive to anakinra

Pediatr Dermatol. 2013 Nov-Dec;30(6):758-60. doi: 10.1111/j.1525-1470.2012.01725.x. Epub 2012 Apr 4.

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.

Publication types

  • Case Reports

MeSH terms

  • Antirheumatic Agents / therapeutic use
  • Female
  • Hereditary Autoinflammatory Diseases / complications
  • Hereditary Autoinflammatory Diseases / drug therapy*
  • Humans
  • Infant
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use*
  • Osteomyelitis / drug therapy*
  • Osteomyelitis / etiology
  • Periostitis / drug therapy*
  • Periostitis / etiology
  • Skin Diseases, Vesiculobullous / drug therapy*
  • Skin Diseases, Vesiculobullous / etiology

Substances

  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein

Supplementary concepts

  • Deficiency of interleukin-1 receptor antagonist