Deficiency of Interleukin‐1 Receptor Antagonist Responsive to Anakinra. Issue 6 (4th April 2012)

• Record Type: Journal Article
• Authors: Schnellbacher, Charlotte; Ciocca, Giovanna; Menendez, Roxanna; Aksentijevich, Ivona; Goldbach‐Mansky, Raphaela; Duarte, Ana M.; Rivas‐Chacon, Rafael
• Is Part Of: Pediatric dermatology. Volume 30:Issue 6(2013)
• Page Start: 758
• Page End: 760
• Publication Date: 2012-04-04
• Subjects: Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/j.1525-1470.2012.01725.x
• Languages: English
• ISSNs: 0736-8046

Abstract

<abstract abstract-type="main" xml:lang="en"> <title> <x xml:space="preserve">Abstract</x> </title> <p> <bold>Abstract: </bold> 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.</p> </abstract>