Autosomal dominant hereditary disorder	Developmental hereditary disorder	Focal facial dermal dysplasia	Hereditary disorder of the integument
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Focal facial dermal dysplasia type I (disorder)
	Focal facial dermal dysplasia type I
	Focal facial dermal dysplasia 1 Brauer type Brauer syndrome FFDD type 1 - focal facial dermal dysplasia type 1 Bitemporal aplasia cutis congenita
	Focal facial dermal dysplasia type I (FFDD1), also known as Brauer syndrome, is a focal facial dysplasia with characteristics of congenital bitemporal cutis aplasia. The bitemporal rarely unilateral hypoplastic scar-like lesions in FFDD, resembling forceps marks, are usually the only manifestations of FFDD1. Most patients usually have normal intelligence. Transmitted in an autosomal dominant manner with full penetrance.
	focale faciale dermale dysplasie type I
	FFDD type 2 bitemporale aplasia cutis congenita syndroom van Brauer

Id	789157007
Status	Primitive

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

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

ICD-10 complex map reference set

Target	Q82.8
Rule	TRUE
Advice	ALWAYS Q82.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified