Cerebral atrophy	Congenital anomaly of cerebrum	Congenital degeneration of nervous system	Follicular keratosis	Hereditary acantholytic dermatosis	Hereditary degenerative disease of central nervous system	Multiple system malformation syndrome	Short stature disorder	X-linked recessive hereditary disease
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Keratosis follicularis, dwarfism, cerebral atrophy syndrome (disorder)
	Keratosis follicularis, dwarfism, cerebral atrophy syndrome
	Syndrome that is characterized by generalized keratosis follicularis, severe proportionate dwarfism and cerebral atrophy. It has been described in six males from one family (three boys and three maternal uncles). Generalized alopecia and microcephaly were also present.
	syndroom van keratosis follicularis, dwerggroei en cerebrale atrofie
	keratosis follicularis-dwerggroei-cerebrale atrofie-syndroom

Id	723830005
Status	Primitive

Associated morphology	Atrophy
Finding site	Cerebrum
Occurrence	Congenital
Pathological process	Pathological developmental process

Has interpretation	Abnormal
Interprets	Keratinization

Associated morphology	Acantholysis
Finding site	Skin structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Follicular hyperkeratosis
Finding site	Hair follicle structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Interprets

Height / growth measure

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q87.1
Rule	TRUE
Advice	ALWAYS Q87.1 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified