Autoinflammatory disease	Autosomal recessive hereditary disorder	Chronic disease of immune structure	Generalized pustular psoriasis	Hereditary disorder of immune system	Hereditary disorder of the integument	Primary immune deficiency disorder
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Deficiency of interleukin 36 receptor antagonist (disorder)
	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.
	deficiëntie van interleukine-36-receptorantagonist
	IL-36Ra-deficiëntie

Id	784339002
Status	Primitive

Associated morphology	Inflammatory morphology
Finding site	Skin structure
Pathological process	Abnormal immune process

Associated morphology	Vesiculobullous rash
Finding site	Skin structure
Pathological process	Hypersensitivity process

Clinical course

Recurrent acute

Associated morphology	Inflammatory morphology
Finding site	Structure of immune system
Pathological process	Abnormal immune process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

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