Bad hair daze: A beginner’s guide to uncombable hair syndrome

I’m sure some of you reading the title of this blog will be thinking that I have made ‘uncombable hair syndrome’ (UHS) up as a way to describe London Mayor Boris Johnson. But it’s not April Fool’s Day and I’m not joking. UHS occurs as the result of a structural deformity in the hair itself and is very rare. UHS goes by a number of different names including ‘pili trianguli et canaliculi’, ‘spun-glass hair’, and ‘cheveux incoiffables’. The condition was first written about scientifically in the 1970s (although earlier reports of the condition had been reported but was not labelled as UHS).

For instance, a case matching contemporary descriptions of UHS by Dr. A.F. Le Double and Dr. F. Houssay was published in 1912. However, it is generally agreed that the syndrome was first formally identified forty years ago (in 1973), by Dr. Dupré, P. Rochiccioli and Dr. J.L. Bonafé in a French paper that described the condition as ‘cheveux incoiffables’. In the following year, two different English speaking doctors (Dr. J.D. Stroud and Dr. A.H. Mehregan) coined the called it ‘spun-glass hair’ in the published proceedings of the ‘First Human Hair Symposium’. By the 1980s, the condition was re-named UHS.

The syndrome is usually noticeable in childhood. Although most parents notice it in their children during their pre-school years, some parents have noticed it a few months after birth but also as late as 12 years of age. A description of UHS can be found in the Wikipedia entry on the condition:

“The hair is normal in quantity and is usually silvery-blond or straw-colored. It is disorderly, it stands out from the scalp, and cannot be combed flat. The underlying structural anomaly is longitudinal grooving of the hair shaft, which appears triangular in cross section. There usually is no family history, though the characteristic hair shaft anomaly can be demonstrated in asymptomatic family members by scanning electron microscopy. To be noticeable, 50% of hairs must be affected by the structural abnormality. Improvement often occurs in later childhood. An autosomal dominant mode of inheritance has been suggested though an autosomal recessive pattern with varying degrees of penetrance has also been noted. The stiffness of the uncombable hair has been reasoned to be due to the triangular form of the hair shaft in cross section. It has been suggested that the condition may result from premature keratinization of the inner root sheath”.

There are also a number of medically recognized conditions where sufferers have the characteristics of UHS. This includes those with ectodermal dysplasia syndrome (these are heritable conditions in which there are abnormalities of two or more ectodermal structures such as the hair, teeth, nails, sweat glands, cranial-facial structure, digits and other parts of the body), retinal dysplasia/pigmentary dystrophy, juvenile cataracts, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia. Both inherited (autosomal dominant and recessive with variable levels of penetrance) and sporadic forms of UHS (i.e., genetic are unclear) have been identified in the medical literature. Most academic papers note that UHS alone is not associated with neurologic, physical, or mental abnormalities. Most of the academic papers in the medical literature are single case studies. Here are a few typical cases that I have found in various medical journals:

• Case 1: “A 4-year-old boy was noted to have unruly, spangled hair, which could not be combed flat. His mother reported that his hair had always had that texture and that it seemed to grow slowly. A hair pull test demonstrated that hairs could not be easily extracted, and light microscopic examination of the hair revealed pathognomonic characteristics of uncombable hair syndrome, including a triangular cross-sectional shape and canal-like longitudinal depressions” (Pediatric Dermatology, 2007).
• Case 2: “The case was a 5-year-old girl. Her whole hair became sparse, and it became a condition, which was immediately formed, when it was held, and the decompression was done. One piece was a condition of curly hair. The scanning electron microscope view showed the pit, which was gentle in the length in paralleling the hair shaft with the hair of about the half. The cross section of a part of hair showed triangle or kidney-shape of which the angle was round. Thus she was diagnosed with uncombable hair syndrome” (Rinsho Derma, 2004).
• Case 3: 
”The patient is a 7 year old Chinese girl who was born vaginally at full term…There was no significant perinatal events and her developmental milestones were normal. She presented with coarse hair since birth, associated with multiple white hairs. There was difficulty with combing. There was no brittleness of hair and no skeletal, nail or skin abnormalities. There was no family history of note…On examination, her hair was coarse, especially over the frontal area of the scalp. There were multiple white hairs interspersed among black hairs. The scalp was normal. The rest of the skin, teeth and skeletal system were normal. Samples of her hair were examined. Under light microscopy the hair shaft appears normal. Under scanning electron microscopy, a longitudinal gutter was seen running the length of the hair shaft. Cross sections of the hair shafts appear triangular or elliptical in shape” (Bulletin For Medical Practitioners, 1995).
• Cases 4-7: “Four children had short, unmanageable, pale blond hair. They had no associated abnormalities and no family histories of abnormal hair. Light microscopy of the hair was normal in three patients, with piii torti present in the fourth. Electron microscopy of hairs from all four children revealed longitudinal grooves in the hair shaft, diagnostic of uncombable-hair syndrome” (Pediatric Dermatology, 1987).
• Cases 8-10: “Three children are reported with uncombable hair syndrome, consisting of slow-growing, straw-colored scalp hair that could not be combed flat. The hairs appeared normal on light microscopy but on scanning electron microscopy were triangular in cross section, with canal-like longitudinal depressions. Oral biotin, 0.3 mg three times a day, produced significant improvement after 4 months in one patient, with increased growth rate and with strength and combability of the hair, although the triangular shape remained. The other two patients were unique in having associated ectodermal dysplasia. Their hair slowly improved in appearance and combability over 5 years without biotin therapy” (Journal of the American Academy of Dermatology, 1985).

According to a review by Dr. P. Calderon and colleagues in a 2009 issue of the Journal of the American Academy of Dermatology, less than 100 cases ever have been reported. They also note that there is no definitive treatment, but that most cases improve with the onset of puberty.

Dr Mark Griffiths, Professor of Gambling Studies, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK

Further reading

Calderon, P., Otberg, N., & Shapiro, J. (2009). Uncombable Hair Syndrome. Journal of the American Academy of Dermatology, 61, 512-515.

Hicks, J., Metry, D.W., Barrish, J., & Levy, M. (2001). Uncombable hair (cheveux incoiffables, pili trianguli et canaliculi) syndrome: Brief review and role of scanning electron microscopy in diagnosis. Ultrastructural Pathology, 25, 99-103.

Ishii, N., Mori, O., & Hashimoto, T. (2004). Uncombable Hair Syndrome. Rinsho Derma (Tokyo), 46, 540-541.

Jarrell, A.D., Hall, M.A., & Sperling, L.C. (2007). Uncombable hair syndrome. Pediatric Dermatology, 24, 436-438.

Matis, W.L., Baden, H., Green, R., Boiko, S., Lucky, A. W., Homstein, L., Ashraf, M. & Hood, A.F. (1987). Uncombable‐hair Syndrome. Pediatric Dermatology, 4, 215-219.

Por, A. (1995). Uncombable Hair Syndrome. Bulletin For Medical Practitioners. Located at: http://www.nsc.gov.sg/showpage.asp?id=373

Rochiccioli, D.A. Bonafé J.L. (1973). Cheveux incoiffables: anomalie con genitale des cheveux. Bull Soe Fr Dermatol Syph, 80, 111-112.

Rest, E.B., & Fretzin, D.F. (1990). Quantitative assessment of scanning electron microscope defects in uncombable‐hair syndrome. Pediatric Dermatology, 7(2), 93-96.

Shelley, W.B., & Shelley, E.D. (1985). Uncombable hair syndrome: observations on response to biotin and occurrence in siblings with ectodermal dysplasia. Journal of the American Academy of Dermatology, 13(1), 97-102.

Stroud, J.D. & Mehregan, A.H. (1974). Spun glass hair: A clinicopathologic study of an unusual hair defect. In: Brown, A.C. (Ed.) The first human hair Symposium. New York: Medcom Press.