Autosomal dominant hereditary disorder	Developmental hereditary disorder	Ectodermal dysplasia with hair-tooth defects	Hereditary disorder of the integument	Hereditary disorder of tooth
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Uncombable hair, retinal pigmentary dystrophy, dental anomaly and brachydactyly syndrome (disorder)
	Uncombable hair, retinal pigmentary dystrophy, dental anomaly and brachydactyly syndrome
	Bork syndrome Tricho-retino-dento-digital syndrome
	An autosomal dominant ectodermal dysplasia syndrome, with characteristics of uncombable hair syndrome, congenital hypotrichosis and dental abnormalities such as oligodontia or hyperdontia and associated with early-onset cataract, retinal pigmentary dystrophy and brachydactyly with brachymetacarpia. Furthermore, hyperactivity and a mild intellectual deficit have been reported in affected patients.
	syndroom van onkambaar haar, retinale pigmentdystrofie, dentale anomalie en brachydactylie

Id	719910004
Status	Primitive

Associated morphology	Morphologically abnormal structure
Finding site	Hair structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Tooth structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Dysplasia
Finding site	Ectoderm structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

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