Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja Sterile multifocal osteomyelitis with periostitis and pustulosis is a rare, severe, genetic autoinflammatory syndrome characterized by usually neonatal onset of generalized neutrophilic cutaneous pustulosis and severe, recurrent, multifocal, aseptic osteomyelitis with marked periostitis, typically affecting distal ribs, long bones and vertebral bodies. High levels of acute-phase reactants (with no fever associated) and onychosis are frequently observed additional features. Disease data Klasyfikacja Disease Synonimy Autoinflammatory disease due to interleukin-1 receptor antagonist deficiency Choroba autoimmunologiczna z powodu niedoboru antagonisty receptora interleukiny-1 DIRA Niedobór antagonisty receptora interleukiny-1 OMPP DIRA Interleukin-1 receptor antagonist deficiency OMPP Kod ORPHA 210115 Kod OMIM 612852 Kod ICD10 D84.8 Kod ICD11 4A00.Y *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl