Naxos disease[1] (also known as "diffuse non-epidermolytic palmoplantar keratoderma with woolly hair and cardiomyopathy"[1] or "diffuse palmoplantar keratoderma with woolly hair and arrhythmogenic right ventricular cardiomyopathy", first described on the island of Naxos by Dr. Nikos Protonotarios[1]) is a cutaneous condition characterized by a palmoplantar keratoderma.
[1] The prevalence of the syndrome is up to 1 in every 1000 people in the Greek islands.
[2] It has been associated with mutations in the genes encoding the proteins desmoplakin, plakoglobin, desmocollin-2, and SRC-interacting protein (SIP).
[3][4] Naxos disease has the same cutaneous phenotype as the Carvajal syndrome.
[2] Between 80 and 99% of those with Naxos disease will display some of the following symptoms: Ankyrin: Long QT syndrome 4