Naxos disease and Carvajal syndrome: cardiocutaneous disorders that highlight the pathogenesis and broaden the spectrum of arrhythmogenic right ventricular …
N Protonotarios, A Tsatsopoulou - Cardiovascular pathology, 2004 - Elsevier
Naxos disease is a recessive association of arrhythmogenic right ventricular
cardiomyopathy (ARVC) with wooly hair and palmoplantar keratoderma or similar skin
disorder. The clinical and histopathological spectrum of heart disease, molecular genetics
and genotype-phenotype correlation are reviewed in 22 affected families with this
cardiocutaneous syndrome reported in the literature from Greece, Italy, India, Ecuador, Israel
and Turkey. All patients had the hair and skin phenotype from infancy and developed ARVC …
cardiomyopathy (ARVC) with wooly hair and palmoplantar keratoderma or similar skin
disorder. The clinical and histopathological spectrum of heart disease, molecular genetics
and genotype-phenotype correlation are reviewed in 22 affected families with this
cardiocutaneous syndrome reported in the literature from Greece, Italy, India, Ecuador, Israel
and Turkey. All patients had the hair and skin phenotype from infancy and developed ARVC …
