Uncombable hair syndrome

Scalp hair-uncombable hair syndrome

Uncombable hair syndrome

Uncombable hair syndrome (UHS), also known as pili trianguli et canaliculi, is a rare structural anomaly of the hair shaft characterized by dry, frizzy hair that is impossible to comb flat. This condition typically becomes apparent in early childhood and is usually resolved or significantly improved by adolescence.

Presentation

Children with uncombable hair syndrome usually present with hair that stands out from the scalp and cannot be flattened or styled. The hair is often described as silvery-blond or straw-colored. The characteristic feature of UHS is the triangular or heart-shaped cross-section of the hair shaft, which can be observed under a microscope.

Genetics

Uncombable hair syndrome is often inherited in an autosomal recessive manner, although autosomal dominant inheritance has also been reported. Mutations in the PADI3, TGM3, and TCHH genes have been associated with the condition. These genes are involved in the formation and structure of the hair shaft.

Diagnosis

Diagnosis of uncombable hair syndrome is primarily clinical, based on the characteristic appearance of the hair. Microscopic examination of the hair shaft can confirm the diagnosis by revealing the distinctive triangular or kidney-shaped cross-sections. Genetic testing can identify mutations in the associated genes.

Management

There is no specific treatment for uncombable hair syndrome. Management focuses on gentle hair care practices to minimize damage and breakage. The condition often improves with age, and many individuals experience normal hair texture by adolescence.

Related Conditions

Uncombable hair syndrome can be associated with other ectodermal dysplasias and may occur in conjunction with other hair shaft disorders such as trichorrhexis nodosa and monilethrix.

See Also

• Ectodermal dysplasia
• Trichorrhexis nodosa
• Monilethrix
• Hair shaft disorders