Preferred Label : Naxos disease;
ICD-11 definition : Naxos disease is a recessively inherited condition with arrhythmogenic right ventricular
dysplasia/cardiomyopathy (ARVD/C) and a cutaneous phenotype, characterised by peculiar
woolly hair and palmoplantar keratoderma.;
ICD-11 synonym : Keratosis palmoplantaris with arrythmogenic cardiomyopathy;
Origin ID : 633516876;
Automatic exact mappings (from CISMeF team)
Naxos disease is a recessively inherited condition with arrhythmogenic right ventricular
dysplasia/cardiomyopathy (ARVD/C) and a cutaneous phenotype, characterised by peculiar
woolly hair and palmoplantar keratoderma.