Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare genetic autoinflammatory syndrome characterized by early-onset of repeated episodes of fever, nodular neutrophil-rich panniculitis, arthralgia, and lipodystrophy. Additional reported features include diarrhea, failure to thrive, lymphadenopathy, and vasculitis. Laboratory examination may reveal elevated serum C-reactive protein and leukocytosis with neutrophilia in the absence of infection. Disease data Classification Disease Synonyms ORAS Otulipenia Niedobór OTULIN Zespół autoinflamatoryjny związany z OTULIN OTULIN deficiency OTULIN-related autoinflammatory syndrome Otulipenia ORPHA code 500062 OMIM code 617099 ICD10 code D89.8 ICD11 code - *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