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What Is Uncombable Hair Syndrome?

Medically Reviewed by Dan Brennan, MD on February 22, 2022

Uncombable hair syndrome, or UHS, is a rare condition that typically causes dry, frizzy hair that you can’t comb flat. It usually emerges before age 3 but may show up as late as age 12. It appears to be rare; scientists know of only about 100 cases. But there are likely many more cases that doctors don’t diagnose because the condition often goes away in childhood. Other names for the condition include:

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

What Are the Symptoms?

Symptoms show up between 3 months and 12 years of age in children with straw-

colored or blond-silvery hair. The hair color may develop over time. You typically have a normal amount of hair, but it often grows more slowly. The hair stands out from the scalp and grows in different directions. It may be impossible to comb it flat against the scalp. (The condition only affects the hair of the scalp, not hair on other parts of the body.)

The hair is not usually more fragile than in people who don’t have the condition, but constant brushing or grooming may be more likely to cause damage.

UHS usually occurs on its own, but in some cases it may be linked to other diseases that require medical treatment. These diseases might include:

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

It’s important to find out whether UHS is linked to these and other conditions so that you and your health care team can help form a treatment plan if you need one.

Other symptoms that may happen in some UHS cases include:

  • Coarse hair texture
  • Dry hair
  • White-colored hair
  • Kinky hair
  • Patchy baldness on the scalp

What Causes It?

UHS typically happens on its own because of changes or “mutations” in three genes that give instructions on how to make strands of hair on your scalp. These genes are:

  • PAD13
  • TGM3
  • TCHH

You typically inherit these gene mutations when both your parents carry a copy of the mutation, though your parents may not have the condition (autosomal recessive inheritance). Scientists think that some people may also get the condition when only one parent carries a mutation (autosomal dominant inheritance).

These mutations change the shape of the hair shaft from tubular to more angular like a triangle or heart shape. This changes the texture, makes it harder to brush, and causes the light to reflect in a way that gives the hair a certain color.

In some cases, people get UHS without mutations in these genes. Scientists continue to study the cause of UHS in these cases.

How Do Doctors Diagnose UHS?

Your doctor will look for typical symptoms of UHS like hair color, texture, and wild growth patterns. They will look at the hair shaft under a microscope for the telltale triangular or kidney shape with a long groove that runs along its length.

There soon may be genetic tests available for UHS.

How Do Doctors Treat UHS?

There is no cure for UHS, but it usually gets better or goes away completely around the start of puberty. Doctors typically suggest that you are gentle with your hair if you have UHS. That means:

  • Use soft brushes.
  • Avoid perms, hair relaxers, and other harsh treatments.
  • Avoid over-brushing your hair.
  • Keep blow-drying your hair to a minimum.

Some people think that biotin supplements can improve the appearance of your hair if you have UHS. More studies are needed to be sure. Talk to your doctor before you start yourself or your child on any type of medication or supplement.

Outlook

UHS is not serious on its own, though in rare cases, it may be a symptom of a more serious condition, which is why you should talk to your doctor if you suspect UHS in yourself or your child.

In general, you won’t need treatment of any sort and the condition will go away on its own in the teen years.

Show Sources

SOURCES:

MedlinePlus: “Uncombable hair syndrome.”

National Organization for Rare Disorders: “NIH GARD Information: Uncombable hair syndrome.”

Genetic and Rare Diseases Information Center (GARD): “Uncombable hair syndrome.”

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