woolly hair syndrome


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.