atopisch eczeem	autosomaal recessieve hereditaire aandoening	chronische aandoening van immuunfunctie	chronische huidziekte	chronische mucocutane candidiase	gecombineerde immunodeficiëntie	hereditaire aandoening van immuunsysteem	hereditaire aandoening van integumentum	verhoogd immunoglobuline
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Autosomal recessive hyperimmunoglobulin E syndrome due to zinc finger protein 341 deficiency (disorder)
	Autosomal recessive hyper-IgE syndrome due to ZNF341 deficiency
	Autosomal recessive hyperimmunoglobulin E syndrome due to zinc finger protein 341 deficiency AR-HIES (autosomal recessive hyperimmunoglobulin E syndrome) due to ZNF341 deficiency
	A rare hyper-IgE syndrome with characteristics of atopic dermatitis (eczema), chronic mucocutaneous candidiasis, and elevated IgE levels due to ZNF341 deficiency. High plasma levels of IgG and low natural killer (NK) cell numbers are observed. Other major clinical features involve recurrent skin infections with skin abscesses and connective tissue abnormalities. Some patients may have recurrent lung infections.

Id	1342372000
Status	Primitive

Clinical course

Associated with

Pathological process

afwijkend immuunproces

Has interpretation	boven referentiebereik
Interprets	bepalen van immunoglobuline E

Associated morphology	inflammatoire morfologie
Finding site	structuur van huid

Causative agent	genus Candida
Finding site	structuur van huid en/of slijmvlies
Pathological process	infectieus proces

SNOMED CT to ICD-10 extended map

Target	D82.4
Rule	TRUE
Advice	ALWAYS D82.4 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified