Autosomal recessive hereditary disorder	Congenital alopecia	Congenital ectodermal defect	Developmental hereditary disorder	Hereditary palmoplantar keratoderma
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Autosomal recessive palmoplantar keratoderma and congenital alopecia syndrome (disorder)
	Autosomal recessive palmoplantar keratoderma and congenital alopecia syndrome
	Autosomal recessive palmoplantar keratoderma and congenital alopecia Wallis type
	Autosomal recessive palmoplantar hyperkeratosis and congenital alopecia (PPK-CA) is a rare genetic skin disorder characterized by congenital alopecia and palmoplantar hyperkeratosis. It is usually associated with cataracts, progressive sclerodactyly and pseudo-ainhum.

Id	719104003
Status	Primitive

Associated morphology	Absence
Finding site	Hair structure
Occurrence	Congenital
Pathological process	Pathological developmental process

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

Associated morphology	Hyperkeratosis
Finding site	Skin structure of sole of foot

Associated morphology	Hyperkeratosis
Finding site	Skin structure of palmar area of hand

SNOMED CT to Orphanet simple map

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q82.8
Term	Overige gespecificeerde congenitale misvormingen van huid

Target	Q84.0
Term	Congenitale alopecia

SNOMED CT to ICD-10 extended map

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

Target	Q84.0
Rule	TRUE
Advice	ALWAYS Q84.0
Correlation	SNOMED CT source code to target map code correlation not specified