woolly hair syndrome


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Tight curly hair seen at birth in Caucasians that is
(1) Sporadic and associated with woolly hair nevus
(2) Autosomal recessive [MIM 278150], in which the scalp hair is ash-white and body hair short and kinky
(3) Autosomal recessive [MIM 601214], a disorder characterised by palmoplantar keratoderma and other ectodermal features with arrhythmogenic right ventricular dysplasia/cardiomyopathy, first reported in a cohort on the Greek island of Naxos
(4) Autosomal dominant [MIM 194300], in which the scalp hair is also ash-white, but with normal body hair

woolly hair syndrome

A rare congenital disorder in which infants are born with wiry or unusually curly hair; sometimes associated with abnormal heart development; sometimes found only on hair that grows on nevi.
References in periodicals archive ?
Woolly hair syndrome. Orphaned Encyclopedia, Dec 2003: 1-4.
[2,3] Woolly hair can appear as a part of systemic disease (Woolly hair syndrome) or without systemic findings (non-syndromic woolly hair).