| deficiëntie van interleukine-36-receptorantagonist (aandoening) | | deficiëntie van interleukine-36-receptorantagonist | | IL-36Ra-deficiëntie
| | Deficiency of interleukin 36 receptor antagonist | | DITRA - deficiency of interleukin 36 receptor antagonist
Deficiency of IL-36Ra (interleukin 36 receptor antagonist)
Deficiency of IL-36R antagonist
| | 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 (for example pitting and onychomadesis) and ichthyosis may occasionally be associated. |
| | Id | 784339002 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | L40.1 | | Term | Gegeneraliseerde psoriasis pustulosa |
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| SNOMED CT to Orphanet simple map | 404546 |
| SNOMED CT to ICD-10 extended map | | Target | L40.1 | | Rule | TRUE | | Advice | ALWAYS L40.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | | Correlation | SNOMED CT source code to target map code correlation not specified |
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