[6] UHS has several names, including pili trianguli et canaliculi (Latin), cheveux incoiffables (French), and "spun-glass hair".

Based on the current scientific studies related to the disorder, the three genes that have been causally linked to UHS are PADI3, TGM3, and TCHH.

These genes code for proteins involved in hair shaft formation and improvement often occurs in later childhood.

[3][7][10] The hair of people with UHS may eventually lie flat and appear near normal in texture by adolescence or early adulthood for unknown reasons.

[3] Basmanav et al. have suggested that either different, non-mutated isoforms of the hair-forming enzymes are produced as we age, or that aging-related mechanistic changes in hair such as greater diameter and length help resolve UHS.

[7] As one would expect from the name, individuals affected by this disorder have difficulty managing and grooming their hair.

Affected individuals usually possess blonde or silver hair that may be present at birth or develop over time.

Trichohyalin (TCHH) is a structural protein responsible for the proper cylindrical fiber topology of the hair shaft.

One study reported PAD involvement in skin and skin-related diseases, particularly at the hair follicle.

The trichohyalin gene is produced and changed by other proteins and to molecules such as keratin intermediate filaments to form organized cross-links.

[16] With the 58 pairs of tissues that were sampled, it was confirmed that transglutaminase 3 is closely related to growth proliferation and migration.

This came to show that transglutaminase 3 will be a candidate growth and could function as a helpful biomarker and a therapeutic target to esophageal cancer treatment.

With immunoblotting, it appeared that the transfected cells had a marginally lower expression of the mutant compared to the wild-type.

[19] Therefore, it helped conclude that there was a reduction in enzymatic action that encodes a protein to hair shaft arrangement, which relates to central centrifugal cicatricial alopecia (CCCA).

However, unlike these conditions, uncombable hair syndrome alone is not associated with physical, neurologic, or mental abnormalities.

[9] SEM allows for the visualization of hair shaft cross-sections, which can appear triangular, heart-shaped, kidney-shaped, flat, or longitudinally grooved.

Interestingly, multiple different cross sections can appear on a single shaft of hair.

[7][3] UHS has been found in patients who also have diseases such as ectodermal dysplasia and loose anagen hair syndrome.

[7] The syndrome's name comes directly from its abnormal, frizzy appearance that "totally resists any effort to control it with brush or comb".

[21] The syndrome was described in 1973 by A. Dupré, P. Rochiccioli, and J. L. Bonafé, who named it cheveux incoiffables (unstyleable hair).

A children’s story published in Germany in 1845 detailed a character named Struwwelpeter or "Shockheaded Peter", presumably to describe the frizzy nature of a patient's hair with UHS.

In 1983, Garty et al. described the case of a 2-year-old boy whose father, grandfather, and great-grandfather were all said to have the same condition at a young age—difficulty combing their hair.