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Naxos disease

ORPHA:34217· ICD-10 Q87.8

Definition

A rare arrhythmogenic right ventricular cardiomyopathy (ARVC) and a cutaneous phenotype, characterized by peculiar woolly hair and palmoplantar keratoderma.

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Infancy, Neonatal