autosomaal dominante hereditaire aandoening	ectodermaal syndroom met defect van haren, tanden en zweetklieren	hereditaire aandoening van gebitselement	hereditaire aandoening van integumentum	hereditaire ontwikkelingsstoornis
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Böök-syndroom (aandoening)
	Böök-syndroom
	syndroom van Böök
	Book syndrome
	A rare autosomal dominant ectodermal dysplasia syndrome characterized by premolar aplasia, hyperhidrosis, and premature graying of the hair. Additional features may include a narrow palate, hypoplastic nails, eyebrow anomalies, a unilateral simian crease, and poorly formed dermatoglyphics.

Id	722296002
Status	Primitive

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

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

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

Has interpretation	afwijkend
Interprets	zweten

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q82.4
Term	Ectodermale dysplasie (anhidrotisch)

SNOMED CT to Orphanet simple map

1262

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