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Unmanageable Hair Syndrome Mutation Cause Symptoms Diagnosis UHS

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You may have a bad hair day when the humidity is high. Recall how unmanageable this makes your strands. Unmanageable hair syndrome.

So, imagine having that bad hair day every day. I saw this article last week. A mom wants to get her story out about her son’s condition.

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The son is Locklan Samples who is just 17 months. He is said to be 1 of 100 who has this syndrome. In other words, it’s an extremely rare syndrome.

When the little guy was born his hair was jet black like his mom Katelyn. But she says when he turned 6 months the dark hair became what Lockland’s parents refer to as “peach fuzz”.

People began to take notice of the boy and mom received a message from a stranger on Instagram. According to the article in People, the stranger questioned if Lock had “uncombable hair syndrome?”

Furthermore, Katelyn checked this out with their pediatrician who had not heard of the condition. So, the family was referred to a specialist nearby.

Specimens were taken of the toddler’s hair to view under a microscope. After that the hair structure was observed the confirmation was indeed delivered that it was uncombable hair syndrome.

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Hair Strands and Follicles

As your hair grows it begins with the hair follicle. If this is healthy the proteins that produce your hair build the hair shaft cylindrically. In other words, your hair grows in a round shape.

This degree of roundness determines what your hair comes out looking like. A perfect circle presents straight hair. Therefore, having a natural spiral cycle in the protein production forms curls. As well as the melanin in your skin also provides your natural hair color.

With those having UHS, their hair follicle isn’t built with a cylindrical shape. And there is no influence of melanin for the color. Researchers have acknowledged that their hair is growing with one or more defined channels. As a result, it shows up slower than normal hair.

A sample of the hair strands actually show a triangular or kidney shaped form. So, the hair beneath a microscope focuses similar to spun glass.

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Unmanageable Hair Syndrome Getting to the Roots

It can be unsettling to receive a diagnosis of UHS. And the fact that it is considered an autosomal recessive genetic disorder can be more frustrating. Thus, an autosomal recessive gene is genetically passed down to those with UHS.

And because it’s recessive the gene can be carried with no noticeable symptoms and no prevention from it forming. UHS won’t cause any other health issues however there is evidence of links to other genetic disorders.

Researchers have separated wooly hair, cheveux incoiffables or UHS into three distinct types:

  • UHS1
  • UNC2
  • UHS3

These three forms of UHS seem to have other genetic construction. But they also share similarities.

Each type contains a different protein that could alter the regular cellular construction of the hair. As a result, the abnormal hair strands sticking out from the hair follicles.

What is Uncombable Hair Syndrome (UHS)?

According to JAMA Dermatology UHS was first described in 1973 by Stroud and Mehregan. MedlinePlus says uncombable hair syndrome is a condition where dry, frizzy hair can’t be combed flat. Further, it is a unique disorder involving the hair shaft.

The disorder usually happens in childhood between infancy and the age of three. However, it can develop as late as 12 years of age.

    Other names for UHS:

  • Cheveux Incoiffables
  • Pili trianguli et canaliculi
  • Spun glass hair
  • Unmanageable hair syndrome

Children with this syndrome have light colored hair either blond or a silver color that has a glistening sheen. As for growing it doesn’t go downward, instead it shoots out in different directions from the scalp. Certainly, by its surface you would think it’s fragile or brittle.

But that is said not to be the case. It does grow at a normal or somewhat slower course. With uncombable hair syndrome just the scalp hair is affected.

It is not perceived why however this condition improves with time. And by the person’s teens with UHS the hair can lie flat and have normal or almost normal texture.

More people are likely to have this condition that have not been diagnosed. As adults they could be unaffected but when children they may have had uncombable hair syndrome.

Unmanageable Hair Syndrome Symptoms

There is usually a normal amount of hair that grows in slowly. Hair will protrude out from the scalp in different directions. In other words, the hair is unmanageable and difficult to comb flat against the scalp.

UHS affects the hair only on the scalp and doesn’t involve hair on other areas of your body. The hair is often not more fragile than those without the condition. However, brushing continually or grooming can cause damage.

sm lt browngraphics

This condition normally shows on its own. On the other hand, it could be linked to other diseases that require treatment.

Diseases UHS could include:

  • Ectodermal dysplasias
  • Bork syndrome
  • Angel-shaped phalangoepiphyseal dsyplasia

Most importantly, identifying whether UHS is connected to a condition will aid to determine a treatment plan.

Other symptoms could be:

  • Coarse hair texture
  • Dry hair
  • White colored hair
  • Kinky hair
  • Patchy bald spots on the scalp

Unmanageable Hair Syndrome the Cause

Unmanageable hair syndrome is generally caused by shifts or mutations in three genes. These provide instructions on how to create your hair strands on your scalp.

Typically, you inherit these gene mutations when your mom and dad carry a duplicate of the mutation. Even though, they don’t have the condition (autosomal recessive inheritance/pattern). However, some individuals can obtain the syndrome if just one parent has a mutation (autosomal dominant inheritance/pattern).

Mutations alter the shape of hair shaft from tubular to more angular similar to a triangle or heart shape. For instance, this changes the texture making it difficult to brush. In addition, light reflected in a way that hair is distributed a certain color.

People in some cases receive UHS without these gene mutations. Scientists are still studying the cases of UHS.

Mutations

Unmanageable hair syndrome is caused by mutations of specific genes.

These genes are:

  • PAD13
  • TGM3
  • TCHH

Proteins made from genes PAD13 and TGM3 alter the protein made by the TCHH gene, called trichohyalin. Therefore, altered tricholyalin can bind with other trichohyalin proteins along with molecules called keratin intermediate filaments to produce organized cross-links.

The cross-links create dense networks. Networks give structure to your hair strand as well as a cylindrical shape.

Mutated genes; PAD13, TGM3 or TCHH likely go to the forming of proteins with 1 minimum or zero activity. Thus, there are adjustments in the hair shaft. That is to say, other than a cylindrical shape there is a triangular heart shape or flat cut over part.

Due to this angular shape with the hair shaft, it can’t lay flat. With children having uncombable hair syndrome, 50 to 100 percent have irregular shaped strands of hair. In addition, the glistening sheen is because abnormal hair reflects light differently compared to normal hair. However, there are individuals without an identified mutation in any of these three genes.

Inheritance

Unmanageable hair syndrome when it is caused by the mutations of both gene is inherited in an autosomal recessive pattern. Meaning both duplicates of the gene have mutations in each cell. Further, the individual’s parents with an autosomal recessive pattern each have a duplicate of the mutated gene. Usually, they don’t show signs or symptoms of the condition.

With other cases, it seems to be inherited in an autosomal dominant pattern.  This is only a copy of the altered gene in each cell which is enough to cause the disorder. Thus, in these situations the affected individual typically inherits mutation from an affected parent. But there is no way of knowing where the connected gene came from.

Even with other cases of uncombable hair syndrome, the inheritance pattern is not known.

How is UHS Diagnosed?

Your dermatologist will be observing usual symptoms of UHS such as hair color, texture and disorderly patterns. The hair is split in half and will be viewed under a high-powered scanning electron microscope. Further, telltale signs of a triangular or kidney shape accompanied by a long groove from root to the end.

In the near future there could be genetic testing available for UHS.

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How is UHS Treated?

UHS has no cure. However, it normally improves or totally goes away when puberty begins. Physicians generally tell you to be gentle with your hair if you have this condition.

Suggestions are:

  • Use soft brushes
  • Don’t get perms or hair relaxers and avoid other harsh treatments
  • Avoid brushing the hair too much
  • Limit blow drying the hair

Biotin

Biotin supplements have been known to improve the development of hair. Some people think this may work if you have UHS. Above all, more studies are required on this. Consult your dermatologist before you or your child begins taking any type of medication or supplement.

Wrapping it up

Uncombable hair syndrome is not a serious condition alone. In rare cases it could be connected to a more serious condition. Most importantly, if you suspect you or your child has UHS visit your dermatologist.

Usually, treatment is not required. And the condition vanishes on its own by adolescence.

Mary

Mary is the founder of All About Our Skin. Former esthetician and CPC. Enjoys researching skincare and has been studying our skin for the past fifteen years.

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