Orphanet: Search a disease

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Naxos disease

Disease definition

A recessively inherited condition with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and a cutaneous phenotype, characterised by peculiar woolly hair and palmoplantar keratoderma.


Classification level: Disorder
  • Synonym(s):
    • KWWH type I
    • Keratoderma with woolly hair type I
    • Keratosis palmoplantaris with arrythmogenic cardiomyopathy
    • Palmoplantar hyperkeratosis with arrythmogenic cardiomyopathy
    • Palmoplantar keratoderma with arrythmogenic cardiomyopathy
  • Prevalence: -
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q87.8
  • OMIM: 601214
  • UMLS: C1832600
  • MeSH: C538346
  • GARD: 9795
  • MedDRA: -

Detailed information


The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.