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Uncombable Hair Syndrome

Léa Prague on February 3, 2023 0 Comments • Tags: #uncombablehairsyndrome

uncombable hair syndrome

Uncombable hair syndrome is a very rare and little known disease. Generally it manifests during childhood and can occur between 3 months and 12 years old.
When this pathology manifests in children, the hair then becomes messy and dry, and gradually changes color to become very light blond, like straw.

The hair is indomitable and goes in all directions. It then becomes totally impossible to style them, or even to flatten them. This syndrome is not physically disabling and these effects fade as the child grows.

Discovered in 1973 by Toulouse dermatologist Michel Simon, uncombable hair syndrome is also known as “Pili trianguli and caniculi“, and is thought to affect approximately one in 10 million people worldwide.

What is Uncombable Hair Syndrome?

To go into specifics, uncombable hair syndrome is actually a rare genetic disorder of the hair shaft. The hair then changes texture and color. It becomes dry, frizzy and straw-blonde. The locks of hair then become unmanageable. Despite all the efforts, the hair is messy and stays that way. Impossible to comb, impossible to flatten, impossible to flatten… This genetic disorder only affects the hair, and not the hair of the rest of the body.

uncombable hair syndrome-1

The structure of the hair shaft

Now that we know that this pathology is a disorder of the hair shaft, it is important to understand how it is composed.

The structure of the hair shaft:

  • The cuticle: outer part of the hair. The cuticle is formed of flattened keratinized cells without any pigment.
  • The cortex: main structure of the hair. The cortex is responsible for providing elasticity and strength to the hair.
  • The marrow: inner part of the hair. The pith has no relation in the stem alterations.

Alterations in the structure of the shaft, and in particular the cuticle and the cortex, can therefore lead to the rare syndrome of unmanageable hair. The hair remains strong but is nevertheless completely untamable due to alterations in the structure of the hair shaft.
Under the microscope, the hair of a person with uncombable hair syndrome is triangular or kidney-shaped. This alteration is declared as uncombable hair syndrome when at least 50% of the follicles are affected.

Is uncombable hair syndrome common?

Uncombable hair syndrome is very rare. So much so that only a hundred cases have so far been reported. In reality, this number is surely higher, but many people do not consult their doctor, because in fact, so far no cure exists.
Signs of this rare genetic disease usually appear between 3 months and 12 years old. It is a pathology that can be hereditary and transmitted from generation to generation.

What Causes Uncombable Hair Syndrome

The causes of unmanageable hair syndrome are mainly its genetic mutations in the following genes: PADI3, TGM3 and TCHH.

These three genes are involved in the formation of the hair shaft. Due to these genetic changes, people with this disorder will therefore have more fragile and brittle hair.
Uncombable hair syndrome can be identified through genetic testing as well as microscopic hair examination. The hair affected by the genetic mutation is easily recognizable: under the microscope it appears in the shape of a triangle or strongly grooved over the entire length of the hair.

What is the cure for uncombable hair syndrome?

Unfortunately, there is no cure for unmanageable hair syndrome. To date, there is no treatment for this very rare condition. Indeed, hair shaft disorders are programmed in the very genetics of the affected person. However, it turns out that the symptoms improve as the child grows.

It is therefore at the start of adolescence that the symptoms will gradually decrease and the hair will then gradually regain a normal texture. Indeed, when entering puberty, many internal and hormonal changes then take place, and in particular at the level of the hair follicles (hair and body hair).
And even though studies conducted in 2007 showed the benefits of the vitamin Biotin, in improving hair texture, the lack of evidence due to the rare cases of this disease, does not make it a reliable remedy. .

The best remedy for uncombable hair syndrome is therefore patience!

How to successfully manage unmanageable hair

Brushing hair with uncombable hair syndrome is not easy, as the name suggests!

First of all it is important to take great care of this hair which in practice is very damaged. It is therefore advisable to use hair masks or keratin-based treatments as often as possible. The hair must be hydrated and deeply nourished.
When brushing, it is important to use a brush or a comb with very soft bristles so as not to break the hair. Finally, it is possible to apply gel or styling mousse to try to tame the hair. Even if the stain turns out to be quite difficult, the hair can also be tied in a braid or a ponytail. Also, the natural look, hair in the wind can also be adopted 🙂




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