Naxos disease

MeSH: C538346ORPHA: 34217

Overview

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

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Naxos disease, sourced from HPO and Orphanet clinical annotations.

Palmoplantar keratodermaCardiomyopathyWoolly hairVertigoParoxysmal ventricular tachycardiaAbnormality of hair textureArrhythmiaCleft upper lipHyperhidrosisCongestive heart failureSparse scalp hairCurly hairAcanthosis nigricansSudden cardiac death

Classification & Codes

MeSH Code

C538346

Orphanet Code

ORPHA:34217
Naxos disease
MeSHC538346
OrphanetORPHA:34217
Treatments0 drug(s)
Symptoms on record14 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO