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Uncombable Hair Syndrome

Rare condition which may occur sporadically or show an autosomal dominant inheritance. The children present in infancy up to puberty with slow-growing, blonde, dry and frizzy hair that is disorderly and unmanageable. Under the scanning electron microscope, longitudinal grooving is seen in conjunction with a cross-sectional triangular shape. Premature keratinization of the inner root sheath may be one possible explanation for the hair shaft abnormality. The condition may improve with age.

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