Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Naxos disease
Introduction
Overview
Overview
Naxos disease is an autosomal recessive variant of ARVD, described initially on the Greek island of Naxos. There, the penetrance is >90%.
It involves the gene that codes for plakoglobin (a protein that is involved in cellular adhesion), on chromosome 17p.
Naxos disease is described as a triad of ARVD, palmoplantar keratosis, and wooly hair.
The signs of Naxos disease are more severe than with autosomal dominant ARVD.
References
References
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]