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Year : 2008  |  Volume : 74  |  Issue : 1  |  Page : 50--52

Naxos disease: A rare occurrence of cardiomyopathy with woolly hair and palmoplantar keratoderma

1 Department of Dermatology, PSG Hospitals, Peelamedu, Coimbatore, India
2 Department of Cardiology, PSG Hospitals, Peelamedu, Coimbatore, India

Correspondence Address:
R Rai
Department of Dermatology, PSG Hospitals, Peelamedu, Coimbatore - 641 004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0378-6323.38410

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Naxos disease is a rare genodermatosis with woolly hair, keratoderma of palms and soles and cardiomyopathy. A seven-year-old boy presented with woolly hair and hyperkeratotic lesions on the palms and soles since birth. His cardiac status was evaluated and echocardiography revealed early cardiomyopathy. Scalp biopsy revealed hair shaft in an angulated outline suggestive of woolly hair. So the diagnosis of Naxos disease was made. Since he was asymptomatic no treatment was offered but a regular follow-up of the patient and treatment of emergent symptoms should prevent sudden death.


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Online since 15th March '04
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