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Cardiovascular Therapy: Open Access
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  • Case Report   
  • Cardiovasc Ther 2016, Vol 1(1): 103

Carvajal Syndrome: A Rare Variant of Naxos Disease

Madhu KJ1*, Vijyalakshmi IB2, Chitra Narsimhan2 and Manjunath CN1
1Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, , India
2Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, , India
*Corresponding Author : Madhu KJ, Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, India, Tel: +9902783016, Email: [email protected]

Received Date: Dec 24, 2015 / Accepted Date: Jan 18, 2016 / Published Date: Jan 25, 2016

Abstract

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.

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