Woolly Hair Disease

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

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woolly hair disease

Pediatrics Tight curly hair seen at birth in Caucasians that is 1. Sporadic and associated with woolly hair nevus 2. AR, in which the scalp hair is ash-white and body hair short and kinky or 3. AD, in which the scalp hair is also ash-white, but with normal body hair. Cf Kinky hair disease, Uncombable hair syndrome.