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deficiëntie van interleukine-36-receptorantagonist (aandoening)
deficiëntie van interleukine-36-receptorantagonist
IL-36Ra-deficiëntie
Deficiency of interleukin 36 receptor antagonist
Deficiency of IL-36R antagonist
DITRA - deficiency of interleukin 36 receptor antagonist
Deficiency of IL-36Ra (interleukin 36 receptor 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.
Id784339002
StatusPrimitive
Associated morphologyinflammatoire morfologie
Finding sitestructuur van huid
Pathological processafwijkend immuunproces
Associated morphologyvesiculobulleus exantheem
Finding sitestructuur van huid
Pathological processproces van overgevoeligheid
Clinical courseacuut recidiverend
referentieset met complexe 'mapping' naar ICD-10
TargetL40.1
RuleTRUE
AdviceALWAYS L40.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified