This healthy 21-month-old girl had frizzy hair since birth and she seldom needed haircuts. She had normal teeth, fingernails, and toenails, and family history was negative for similar hair problems. Uncombable hair syndrome was first reported in 1973 by Dupré. Microscopically, hairs are triangular in shape with longitudinal grooves, a finding known as pili trianguli et canaliculi. This hair shaft abnormality is noted in the first few years of life as dry, light blond hair that projects straight from the scalp and cannot be combed. The hair grows slowly and infrequent haircuts are needed. The condition is thought to have an autosomal dominant inheritance pattern, but most cases are sporadic. Uncombable hair is not usually associated with ectodermal defects. There is no known definitive therapy, although there have been case reports of spontaneous improvement in late childhood as well as anecdotal responses to biotin supplementation.
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