Cheveux incoiffables

From WikiMD.com Medical Encyclopedia

Cheveux Incoiffables Cheveux incoiffables, also known as Uncombable Hair Syndrome (UHS), is a rare structural anomaly of the hair shaft characterized by dry, frizzy hair that is impossible to comb flat. This condition typically presents in childhood and is often associated with a distinctive silvery-blond or straw-colored appearance.

Clinical Presentation[edit | edit source]

Individuals with Cheveux incoiffables usually present with hair that stands out from the scalp and cannot be styled or flattened. The hair is often described as having a "spun glass" appearance. The condition is most noticeable in children between the ages of 3 months and 12 years, and it may improve with age.

Genetics[edit | edit source]

Cheveux incoiffables is often inherited in an autosomal dominant manner, although cases of autosomal recessive inheritance have also been reported. Mutations in the PADI3, TGM3, and TCHH genes have been implicated in the development of this condition. These genes are involved in the formation and structure of the hair shaft.

Diagnosis[edit | edit source]

Diagnosis of Cheveux incoiffables is primarily clinical, based on the characteristic appearance of the hair. A microscopic examination of the hair shaft can reveal a triangular or heart-shaped cross-section with a longitudinal groove, which is diagnostic of the condition.

Management[edit | edit source]

There is no specific treatment for Cheveux incoiffables. Management focuses on gentle hair care practices to minimize damage. Some individuals may find that their hair becomes more manageable with age.

Epidemiology[edit | edit source]

Cheveux incoiffables is an extremely rare condition, with only a few hundred cases reported in the medical literature. It affects individuals of all ethnic backgrounds, although it is more commonly reported in Caucasian populations.

See Also[edit | edit source]

External Links[edit | edit source]

NIH genetic and rare disease info[edit source]

Cheveux incoiffables is a rare disease.

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Contributors: Prab R. Tumpati, MD