DITRA

Orpha code: 404546OMIM code: 614204

Definicja

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
Klasyfikacja

Disease

Synonimy
Deficiency of IL-36R antagonist
Niedobór antagonisty IL-36R
Niedobór IL-36Ra
Deficiency of IL-36Ra
Kod ORPHA
404546
Kod OMIM
614204
Kod ICD10
L40.1
Kod ICD11
-

No additional description.

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