Arrhythmogenic right ventricular dysplasia	Autosomal recessive familial wooly hair Autosomal recessive familial woolly hair	Congenital anomaly of foot	Congenital anomaly of hand	Congenital anomaly of myocardium	Congenital palmoplantar keratoderma	Congenital right ventricular anomaly	Hereditary palmoplantar keratoderma
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Keratosis palmoplantaris and arrhythmogenic cardiomyopathy syndrome (disorder)
	Naxos disease
	Keratoderma with wooly hair type I Keratosis palmoplantaris and arrhythmogenic cardiomyopathy syndrome Keratoderma with woolly hair type I Palmoplantar hyperkeratosis with arrhythmogenic cardiomyopathy
	A recessively inherited condition with arrhythmogenic right ventricular dysplasia/cardiomyopathy and a cutaneous phenotype with manifestation of peculiar wooly hair and palmoplantar keratoderma. The disease was first described in families originating from the Greek island of Naxos. Woolly hair appears from birth, palmoplantar keratoderma develops during the first year of life and cardiomyopathy is clinically manifested by adolescence with 100% penetrance. Symptoms of right heart failure appear during the end stages of the disease. A recessively inherited condition with arrhythmogenic right ventricular dysplasia/cardiomyopathy and a cutaneous phenotype with manifestation of peculiar woolly hair and palmoplantar keratoderma. The disease was first described in families originating from the Greek island of Naxos. Woolly hair appears from birth, palmoplantar keratoderma develops during the first year of life and cardiomyopathy is clinically manifested by adolescence with 100% penetrance. Symptoms of right heart failure appear during the end stages of the disease.
	syndroom van palmoplantaire keratodermie en aritmogene cardiomyopathie
	keratosis palmoplantaris met aritmogene cardiomyopathie ziekte van Naxos keratodermie met wollig haar type I KWWH type I

Id	715535009
Status	Primitive

Associated morphology	Hyperkeratosis
Finding site	Skin structure of palmar area of hand
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Hyperkeratosis
Finding site	Skin structure of sole of foot
Occurrence	Congenital
Pathological process	Pathological developmental process

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

Associated morphology	Morphologically abnormal structure
Finding site	Structure of myocardium of right ventricle
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q87.8
Rule	TRUE
Advice	ALWAYS Q87.8
Correlation	SNOMED CT source code to target map code correlation not specified