Uncombable Hair Syndrome

Uncombable hair syndrome is a condition in which the inflexible, brittle, coarse and straw-colored hair fibers grow in all directions, in frizzy patches. Such hair cannot lie flat, when combed, due to the abnormality in its structure. This condition is also known as 'Pili trianguli et canaliculi', 'Spun-glass hair' or 'Cheveux incoiffable'. Uncombable hair syndrome was discovered by French scientists, during the 1970s. They named it 'Cheveux incoiffable'. The name 'Spun-glass hair' is derived from the dry, curly, glossy, blonde or straw-colored appearance of the hair, which resemble threads of spun glass. This condition is very rare, and only 60 cases were reported from the early seventies to the late nineties.

Symptoms
Uncombable hair syndrome occurs mostly in children. It develops shortly after birth, but becomes apparent at any stage between three months to 13 years. Initial stages is characterized by thin and glossy hair, which gradually turn brittle, dry, curly and more lighter in color. It becomes uncontrollable and uncombable, and cannot be chemically relaxed or straightened. It is said that this condition affects scalp hair only. Other symptoms of uncombable hair syndrome are as follows:

• The hair is slow growing, thin, brittle and has very less or no pigment.
• In some cases, partial baldness is also seen. As the hair is not properly attached to the follicle due to its peculiar structure, it is easy to fall off.
• Though, this condition is related to scalp hair only, in some cases, hair in the eyebrows and eyelashes can be sparse.
• As the basic structure of hair is similar to that of teeth and nails, people affected with uncombable hair syndrome may have short, brittle and split nails and teeth with enamel defects and aberrations.

Usually, uncombable hair syndrome is not associated with any physical or mental disorders.

Causes
The main reason for uncombable hair syndrome is said to be the anomaly in the structure of the hair fiber. Each hair fiber has a triangular or irregular-shaped cross section, and one or more longitudinal grooves, which run through the entire length. The other name for this condition - 'Pili trianguli et canaliculi', is derived from this feature. 'Pili' means hair, ' trianguli' for triangular cross section and 'canaliculi' refers to the longitudinal canals. According to some researchers premature keratinization (deposit of keratin, an insoluble protein substance that is the chief structural constituent of hair and nails) of the inner root sheath, can also result in this condition. These features can be diagnosed by scanning electron microscopy of hair fibers.

The affected people may or may not have a family history of uncombable hair syndrome, but some studies suggest an autosomal dominant mode of inheritance and an autosomal recessive pattern, with varying degrees of penetrance. The hair of family members of an affected person, may appear normal, but can have some strands with structural anomaly, which can be detected through scanning electron microscopy. The only difference is that in people with this condition, more than 50% of their hair would be affected, whereas the amount of affected hair is lesser in their normal family members. Hence, to recognize this condition more than 50% of the hair should have structural abnormalities.

Treatment
At present, there is no certified treatment for uncombable hair syndrome, but some reports suggest that daily doses of biotin can result in some improvements. It is said that the regular use of biotin for a period of at least four to five months can make the hair more pliable and easier to comb. This treatment does not change the structural abnormalities of the hair. Some studies suggest that silver nitrate compresses, mycolog ointment, and occasional antibiotics can also be effective. Always remember to consult your doctor before using any such medications. It is also seen that in some cases, this condition is reversed naturally (without any medications), as the children reach adolescence. The hair may become thick and darker in color, with time. Even the texture can change to normal.

If your child has the above said symptoms, make it sure that it is due to uncombable hair syndrome, as many other diseases are also characterized by such symptoms. You can use some hair products to manage the hair, but only after consulting your doctor. May be, the hair can become normal as your child reaches adulthood.