acute bulleuze eruptie van huid	auto-inflammatoire aandoening	autosomaal recessieve hereditaire aandoening	gegeneraliseerde psoriasis pustulosa	hereditaire aandoening van immuunsysteem	hereditaire aandoening van integumentum	primaire immunodeficiëntie	vesiculair exantheem van huid
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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 (e.g. pitting and onychomadesis) and ichthyosis may occasionally be associated.

Id	784339002
Status	Primitive

Associated morphology	inflammatoire morfologie
Finding site	structuur van huid
Pathological process	afwijkend immuunproces

Associated morphology	vesiculobulleus exantheem
Finding site	structuur van huid
Pathological process	proces van overgevoeligheid

Clinical course

acuut recidiverend

Associated morphology	inflammatoire morfologie
Finding site	structuur van immuunsysteem
Pathological process	afwijkend immuunproces

Associated morphology	pustuleus exantheem
Finding site	structuur van huid
Pathological process	proces van overgevoeligheid

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	L40.1
Term	Gegeneraliseerde psoriasis pustulosa

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