Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare, genetic, autoinflammatory syndrome with immune deficiency disease characterized by recurrent and severe flares of generalized pustular psoriasis associated with high fever, asthenia, and systemic inflammation, due to IL36R antagonist deficiency. Psoriatic nail changes (e.g. pitting and onychomadesis) and ichthyosis may occasionally be associated. Disease data Classification Disease Synonyms Deficiency of IL-36R antagonist Niedobór antagonisty IL-36R Niedobór IL-36Ra Deficiency of IL-36Ra ORPHA code 404546 OMIM code 614204 ICD10 code L40.1 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