Carvajal Syndrome: A Rare Variant of Naxos Disease
Received Date: Dec 24, 2015 / Accepted Date: Jan 18, 2016 / Published Date: Jan 25, 2016
Carvajal syndrome also known as ‘Striate palmoplantar keratoderma with woolly hair and cardiomyopathy is a cutaneous condition inherited in an autosomal recessive pattern due to a defect in desmoplakin gene. The skin disease presents as a striate palmoplantar keratoderma particularly at sites of pressure. The patient is at risk of sudden cardiac death due to dilated cardiomyopathy associated with this entity. A variant of Naxos disease,reported as Carvajal syndrome, has been described in families from India and Ecuador. Clinically, it presents with the same cutaneous phenotype and predominantly left ventricular involvement
Keywords: Carvajal syndrome; Naxos disease; Striate palmoplantar; Autosomal recessive keratoderma
Citation: Madhu KJ, Vijyalakshmi IB, Narsimhan C, Manjunath CN (2016) Carvajal Syndrome: A Rare Variant of Naxos Disease. Cardiovasc Ther 1: 103.
Copyright: © 2016 Madhu KJ, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.