Pediatric Dermatology · 2012 · 78 citations · 4 references
MedicineImmunologyPediatricsClinical ImmunologyInflammationPediatric RheumatologyImmune-mediated Inflammatory DiseasesImmunopathology Of SkinInflammatory DermatosesInflammatory ProcessesInflammatory Skin DiseasesInflammatory Skin DisordersAcute Phase ReactantsCytokine-mediated Skin InflammationInflammation In NewbornsInflammatory Skin ResponsesNeutrophilic Dermatoses In InfectionPustular DermatitisPustular LesionsSystemic Treatments
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.
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