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Deficiency of Interleukin-1 Receptor Antagonist Responsive to Anakinra


Address correspondence to Giovanna Ciocca, M.D., Division of Dermatology, Miami Children’s Hospital, 3100 S.W. 62nd Avenue, Miami, FL 33155, USA, or e-mail:


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.