acanthosis nigricans	autosomaal recessieve hereditaire aandoening	ectodermale dysplasie	hereditaire keratinisatiestoornis	hereditaire ontwikkelingsstoornis
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syndroom van ectodermale dysplasie met acanthosis nigricans (aandoening)
	syndroom van ectodermale dysplasie met acanthosis nigricans
	ectodermale dysplasie-acanthosis nigricans-syndroom syndroom van Lelis Lelis-syndroom
	Lelis syndrome
	Ectodermal dysplasia with acanthosis nigricans syndrome
	The association of ectodermal dysplasia (hypotrichosis and hypohidrosis) with acanthosis nigricans. So far, only eight cases have been described in the literature. Other clinical features may include palmoplantar hyperkeratosis, nail dystrophy, intellectual deficit and hypodontia. Transmission is autosomal recessive.

Id	719429003
Status	Primitive

Associated morphology	dysplasie
Finding site	structuur van ectoderm
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Has interpretation	afwijkend
Interprets	keratinisatie

Associated morphology	morfologische afwijking
Finding site	structuur van huid
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q82.4
Term	Ectodermale dysplasie (anhidrotisch)

Target	L83
Term	Acanthosis nigricans

SNOMED CT to Orphanet simple map

140936

SNOMED CT to ICD-10 extended map

Target	Q82.4
Rule	TRUE
Advice	ALWAYS Q82.4 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified

Target	L83
Rule	TRUE
Advice	ALWAYS L83
Correlation	SNOMED CT source code to target map code correlation not specified