Table 1.

Woolly hair-Palmoplantar keratoderma syndromes

	Type 1 (NAXOS syndrome)[1]	Type 2 (CARVAJAL syndrome)[2]	Type 3 (NAXOS-like phenotype)	Type 4
Epidemiology	Presents during 2nd-3rd decade.	Presents early during childhood.	-	-
	1:1000 incidence in Greek islands
Clinical features	diffuse palmoplantar keratoderma, woolly hair, and recessive form of arrhythmogenic right ventricular cardiomyopathy	Striate keratoderma, woolly hair and dilated left ventricular cardiomyopathy.	Arrhythmogenic right ventricular cardiomyopathy, woolly hair and mild palmoplantar keratoderma	Woolly hair with palmoplantar keratoderma without cardiac abnormality
Gene mutation	JUP gene (encodes plakoglobin)	DSP gene (encodes desmoplakin)	DSC2 gene (encodes desmocollin-2)	KANK2 mutation
Prognosis	High risk of sudden cardiac death	Heart failure and sudden cardiac death can occur in adolescence	-	Good prognosis due to absence of cardiac involvement