Uncombable Hair Syndrome: hfinformatics

 

Overview:

Uncombable hair syndrome is an unusual genetic condition that affects the appearance of your child’s hair. It causes a child’s hair to grow in various directions, and hair becomes frizzy, rough, and dry. Uncombable hair syndrome makes brushing or combing hair difficult. There is no treatment available so far, but it can resolve on its own during adolescence. 

Although uncombable hair syndrome may cause concerns, it does not affect a child's overall health. This condition is unique to each individual and does not result in any symptoms that can affect their overall well-being. If you have any concerns or questions regarding the management of uncombable hair syndrome, it is best to discuss them with a healthcare provider.

What is uncombable hair syndrome?

Uncombable Hair Syndrome or pili trianguli et canaliculi is an unusual genetic condition that causes a person's hair to grow in multiple directions, making it rough, frizzy, blond hair, hard to comb or brush, and look like a synthetic doll hair^1,2,^3.

The hair may look shiny because of the way light reflects off the grooved and flattened hair shaft¹. This condition typically gets better on its own during adolescence, and there is currently no known cure for it.

Who does uncombable hair syndrome affect?

Uncombable hair syndrome causes unruly hair that cannot be combed. However, it typically starts getting better during adolescence, usually around the time of puberty. This condition can last into early adulthood. During this period, a child's hair will begin to grow in one direction (downward) instead of in multiple directions, and it may take several years for all of their hair strands to start growing in one direction³.

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How rare is uncombable hair syndrome?

The condition goes away as children age, so the exact rate of occurrence is unknown. There are over 100 cases of uncombable hair syndrome recorded in medical literature, but there likely exist more cases².

How does uncombable hair syndrome affect my child’s body?

Uncombable hair syndrome is an uncommon genetic condition that affects only the hair on a child's scalp. The condition causes hair to grow in various directions instead of downwards, making it challenging to comb or brush it properly². The name "uncombable hair syndrome" is derived from this characteristic of the condition. This condition only affects the hair on the head and does not impact any other parts of the body³.

Symptoms:

At what age do symptoms of

 uncombable hair syndrome show up?

Uncombable hair syndrome typically starts in infancy, and symptoms may appear around age 3. The symptoms can vary for each person, starting in infancy or appearing later in childhood by age 12. As the child enters adolescence and early adulthood, the symptoms slowly improve, and their hair will start to grow down and lay flat².

What are the symptoms of uncombable 

hair syndrome?

Symptoms of uncombable hair syndrome affect the hair on your child's scalp³.

• Dry
• Rough texture
• Shiny
• Frizzy and can’t be combed or brushed smooth or flat
• Light (hypopigmented) in color, or appears silver, white, or blonde to light brown
• Untamed and grows in all directions instead of only growing downward

Not all children diagnosed with this condition will experience all symptoms. For example, the tone or color of a person’s hair could be naturally black or brown but still be uncombable.

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Causes:

What causes uncombable hair 

syndrome?

Uncombable hair syndrome is a genetic condition caused by a mutation in one of the following genes:

• TGM3
• PADI3
• TCHH
• An additional gene has not yet been identified.

These genes provide instructions for the hair strands to grow cylindrical, which helps the hair to grow in one direction out of the hair follicle, similar to how a vase holds a flower to help it grow upright. However, if there's a genetic mutation in any of these genes, it can affect the shape and structure of the hair shaft. Instead of a cylinder, the hair shaft could have an abnormal shape, such as a triangle, octagon, or heart, which can alter the direction in which the strand grows³.

Diagnosis and Tests:

How is uncombable hair syndrome 

diagnosed?

Uncombable hair syndrome is diagnosed through a complete medical history and a physical examination, where the healthcare provider will assess the unique symptoms of the condition by visually examining the hair on the scalp. The characteristic longitudinal grooves along the hair shaft, along with the triangular or kidney-shaped cross-section, can be observed and analyzed under a microscope. This microscopic examination helps in diagnosing uncombable hair syndrome by identifying these distinct features of the hair strands².

What tests diagnose uncombable hair 

syndrome?

Your healthcare provider may conduct tests to confirm a diagnosis of uncombable hair syndrome, including:

1. Hair shaft test: 

Your provider will examine a strand of your child’s hair under a microscope to look for irregular shapes, which can indicate uncombable hair syndrome³.

2. Genetic test: 

Your provider will analyze a small blood sample to check for any changes in your child's genetic code, which can help confirm the diagnosis of uncombable hair syndrome.

Management and Treatment:

How is uncombable hair syndrome 

treated or managed?

There is no specific treatment for uncombable hair syndrome. However, you can make your child's hair care routine easier by:

• Avoiding hair treatments with harsh chemicals like perms or dyes, as they may not work well or could worsen symptoms.
• Do not over-brushing or over-combing your child's hair.
• Limiting the use of tools like a curling iron or a blow dryer on your child's hair.
• Regularly cutting or trimming your child's hair.

Which hair products can help manage 

uncombable hair?

Hair products such as detanglers, conditioners, or hair masks may not be effective for hair affected by uncombable hair syndrome. Stronger chemicals like perms or hair relaxers struggle to adhere to hair strands and seldom provide benefits for individuals with uncombable hair syndrome.

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Prevention:

How can I prevent uncombable hair 

syndrome?

You cannot prevent uncombable hair syndrome because it is caused by a genetic mutation. If you want to understand your risk of having a child with a genetic condition, consider discussing genetic testing with your healthcare provider.

What can I expect if my child has uncombable hair syndrome?

Uncombable hair syndrome is a temporary condition that typically resolves as a child grows into adulthood. While managing their hair may be challenging, consistent haircuts or maintaining a shorter length can make it easier to handle. It's best to avoid perms, straightening chemicals, or other hair treatments that may not work as expected and could potentially damage your child's hair. Their hair will grow at a normal or slightly slower rate than expected. Some children with uncombable hair syndrome may experience low self-esteem due to their hair looking different from their peers. Seeking support from a mental health professional throughout childhood can help improve their self-image.

When should I visit my healthcare provider?

You should visit your healthcare provider if your child is experiencing self-esteem and mental health issues due to their uncombable hair syndrome diagnosis. The symptoms of this condition do not impact your child's overall health and are purely cosmetic.

What questions should I ask my 

healthcare Provider?

Following questions you should ask your healthcare provider if your child has Uncombabale hair syndrome;

• How can I help my child manage their uncombable hair?
• Are there any specific hair care products or treatments that may be beneficial for my child's condition?
• What can I do to support my child's self-esteem and mental well-being concerning their uncombable hair syndrome?

Conclusion:

In conclusion, uncombable hair syndrome is a rare genetic condition that affects the appearance of a child's hair, causing it to grow in various directions and become frizzy, rough, and dry. While there is no specific treatment for this condition, it typically resolves on its own during adolescence. Although it may cause concerns, uncombable hair syndrome does not affect a child's overall health and is unique to each individual. Seeking support from a healthcare provider is recommended if there are concerns or questions regarding the management of uncombable hair syndrome. Additionally, it's important to provide support for a child's self-esteem and mental well-being with their uncombable hair syndrome.