Engelsk navn: DITRA
Engelske synonym: Deficiency of IL-36R antagonist,Deficiency of IL-36Ra


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.

Fra Orphanet

ORPHA: 404546
Klassifiseringsnivå: Disorder
ICD-10: L40.1

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