• Uncombable hair syndrome
• Uncombable hair syndrome references

Uncombable hair syndrome

Uncombable hair syndrome has also been named "spun glass hair". The names both literally describe the hair fiber. The hair looks like spun glass strands growing in any and all directions and extremely difficult to control and maintain a hair style. It was first described clinically in 1973 although the German fairy tale about "Struwwel Peter" whose hair was a mess, or struwwel, (more literally translated as "shock headed") was possibly based on someone with uncombable hair and mental retardation. Uncombable hair syndrome is quite rare with only around 60 cases reported in the journal literature between 1973 and 1998.

The hair fiber is particularly inflexible and that makes it difficult to make it lay flat against the scalp. The fibers' cross section shape can be quite irregular with one or more longitudinal grooves. Scalp hair in uncombable syndrome usually has little or no pigment, very dry, can be brittle, and spangled appearance. The hair is cut infrequently as it is slow growing and can be easy to pull out. Diffuse alopecia is possible because the hair is not always anchored in the hair follicle properly and also the brittle nature of the hair means the hair is easy to break off. Eyebrow and eyelash hairs are usually normal but can be sparse. Problems with nails have been reported in association with uncombable hair syndrome. The nails can be short, brittle, and easy to split. Teeth may have aberrations such as enamel defects. Both nails and teeth have many similarities in basic structure with hair follicles so it is not surprising that they are also affected. Diagnosis is best made by scanning electron microscopy of hair fibers. Light microscopy does not always show up the abnormalities in the hair fibers although polarized light is helpful.

When looking at the hair follicles that produce these hairs they are normal except that the root sheaths also have an irregular groove shape and they are keratinized early. It may be that early keratinization of the sheaths forms a straight, strong tube through which the hair fiber must pass. This inflexible tube forces the shape of the fiber to be straight and inflexible. The hair fiber itself is also highly keratinized with an excess of sulfur protein bonds in the hair structure. This added strength makes the hair inflexible when exposed above the skin. The inflexible hair may be more brittle and easy to break off in some cases.

The abnormality can be familial genetically inherited. It can have an autosomal dominant mode of inheritance. Recently a project looked at hair from family members of an affected child. It was found that other family members had subtle differences in their hair suggesting the child had a more extreme presentation of a condition that had been present in the family for several generations.

Uncombable hair occurs in children most frequently develops shortly after birth but can develop at any time until the age of puberty. For uncombable hair, the hair looks clinically similar to other conditions but should be distinguished from loose anagen hair syndrome, monilethrix, pili torti, Marie-Unna syndrome, progeria, and Menekes' kinky hair syndrome. Conditions reported in association with uncombable hair include atopy (allergies) ectodermal dysplasia, progressive alopecia areata, and ichthyosis vulgaris.

No real treatment has been found although there is the possibility of spontaneous recovery with advancing age. There has been one anecdotal report of treatment with biotin 0.3mg orally three times daily. This was reported to reverse hair fragility and abnormal loss and increase growth rate after 4 months of use - although the triangular appearance of the hairs did not change (Shelly 1985). Hair was more pliable and somewhat easier to comb. Their hair slowly improved in manageability over 5 years from first diagnosis without use of biotin. The article also mentions use of silver nitrate compresses, mycolog ointment, and occasional antibiotics. Some cases of uncombable hair syndrome spontaneously recover after a few years.

Struwwel Peter was the invention of Dr Heinrich Hoffmann, born June 13, 1809, Frankfurt am Main [Germany] died Sept. 20, 1894. Peter first appeared in "Lustige Geschichten und drollige Bilder mit füntzehn schön kolorten Tafeln für Kinder" von 3-6 Jahren (1845; Slovenly Peter; or, Cheerful Stories and Funny Pictures for Good Little Folks). This book of cautionary tales was written as a Christmas gift for the doctor's four-year-old son. The Character Struwwel Peter was probably based on an individual with uncombable hair syndrome and attention deficit disorder. Sieh einmal, hier steht er. Pfui ! Der Struwwelpeter ! An den Händen beiden Ließ er sich nicht schneiden Seine Nägel fast ein Jahr; Kämmen ließ er nicht sein Haar. "Pfui !" Ruft da ein jeder: "Garst'ger Struwwelpeter !" Mark Twain's translation: See this frowsy "cratur" - Pah ! its's Struwwelpeter ! On his fingers rusty, On his tow-head musty, Scissors seldom come; Let his talons grow a year, - Hardly ever combs his hair, - Do any loathe him ? Some ! They hail him "Modern Satyre - Disgusting Struwwelpeter."

Uncombable hair syndrome

• Itin PH, Buhler U, Buchner SA, Guggenheim R. Pili trianguli et canaliculi: a distinctive hair shaft defect leading to uncombable hair. Dermatology. 1993;187(4):296-8.
• Rest EB, Fretzin DF. Quantitative assessment of scanning electron microscope defects in uncombable-hair syndrome. Pediatr Dermatol. 1990 Jun;7(2):93-6.
• Salinas CF, Montes GM. Rapp-Hodgkin syndrome: observations on ten cases and characteristic hair changes (pili canaliculi). Birth Defects Orig Artic Ser. 1988;24(2):149-68.
• Matis WL, Baden H, Green R, Boiko S, Lucky AW, Hornstein L, Ashraf M, Hood AF. Uncombable-hair syndrome. Pediatr Dermatol. 1987 Nov;4(3):215-9.
• Shelley WB, Shelley ED. Uncombable hair syndrome: observations on response to biotin and occurrence in siblings with ectodermal dysplasia. J Am Acad Dermatol. 1985 Jul;13(1):97-102.
• de Luna MM, Rubinson R, de Kohan ZB. Pili trianguli canaliculi: uncombable hair syndrome in a family with apparent autosomal dominant inheritance. Pediatr Dermatol. 1985 Jul;2(4):324-7.
• Braun-Falco O, Ryckmanns F, Heilgemeir GP, Ring J. [A syndrome: uncombable hair. Observation of 6 members of a family with pili canaliculi, associated with pili torti, progressive alopecia, atopic eczema and hamartomas]. Hautarzt. 1982 Jul;33(7):366-72.
• Hebert AA, Charrow J, Esterly NB, Fretzin DF. Uncombable hair (pili trianguli et canaliculi): evidence for dominant inheritance with complete penetrance based on scanning electron microscopy. Am J Med Genet. 1987 Sep;28(1):185-93.
• Zanca A, Zanca A. Ancient observations of "uncombable hair syndrome". Int J Dermatol. 1993 Oct;32(10):707.
• Van Neste D, Baden HP. Abnormal fibrous protein patterns in the uncombable hair syndrome. Arch Dermatol Res. 1985;277(2):151-2.
• Dupre A, Bonafe JL, Litoux F, Victor M. [Uncombable hair syndrome. Pili trianguli et canaliculi]. Ann Dermatol Venereol. 1978 Jun-Jul;105(6-7):627-30.