Deficiency of Interleukin-1 Receptor Antagonist Responsive to Anakinra

Overview

Journal Pediatr Dermatol

Publisher Wiley

Specialties Dermatology
Pediatrics

Date 2012 Apr 5

PMID 22471702

Citations 27

Authors

Charlotte Schnellbacher

Giovanna Ciocca

Roxanna Menendez

Ivona Aksentijevich

Raphaela Goldbach-Mansky

Ana M Duarte

Rafael Rivas-Chacon

Affiliations

Soon will be listed here.

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.

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