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What Is Ambras Syndrome?

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  • Written By: Mary McMahon
  • Edited By: Shereen Skola
  • Last Modified Date: 16 November 2016
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Ambras syndrome is the presence of excessive hair that is not caused by androgens, as is the case with hirsutism. This condition may be congenital or, more commonly, may be acquired. Several treatment options are available to manage Ambras syndrome in cases where it causes distress for a patient, which can be an issue when it covers large areas of the body. A specialist in dermatology may supervise care for complex cases.

Several divisions are used to distinguish between different forms of this condition. Localized forms feature hair growth in a limited area, in contrast with generalized forms, where it covers the whole body. Patients may have lanugo, which is a fine and soft hair, or terminal hair, which is pigmented and fully mature. The type of hair and area of growth can provide important clues about the cause. Finally, doctors can also determine whether a case is inherited, or acquired for other reasons.

People with congenital forms of Ambras syndrome have genetic mutations that cause the abnormal hair growth. This condition can be hereditary, as cases dating back to the middle ages document. It may also appear spontaneously due to random genetic mutations. These patients may show signs shortly after birth, making the condition evident. Acquired Ambras syndrome can be caused by medications and certain illnesses.

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This condition is considered a skin disease, and treatment options can vary. Some patients may seek hair removal with waxing, lasering, or simple shaving to keep the growth to a minimum. Others may benefit from treatment of an underlying medical issue, such as changing medications, to limit the excessive hair. It can take time for medical treatments to take effect, as the body could need time to adjust. During this period, the hair may continue to grow and cause distress.

Cases of Ambras syndrome are quite rare. Historically, some people with this condition made a living in traveling circuses and freak shows by displaying their bodies to members of the public. This condition can also be associated with dental abnormalities, and as a result it is sometimes referred to as werewolf syndrome. Some carnival performers capitalized on this with nicknames like “The Dog-faced Boy,” drawing attention to their distinctive teeth and gums.

In cases where dental abnormalities cause speech or eating problems, a dentist may be involved in treatment. Patients could benefit from surgery or braces to straighten teeth and address overgrowth of the gums. It may also be important to monitor the mouth on an ongoing basis for signs of complications.

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bear78
Post 3

@burcidi-- Ambras syndrome is actually a sub-type of hypertrichosis syndrome. All hypertrichosis types are characterized by facial and body hair. The difference with Ambras syndrome is that the hair grows very long and straight in a light-blondish color and is very persistent.

There is another sub-type of hypertrichosis, for example, that grows short and curly hair that is dark in color. This is actually the type where oversized gums and teeth are often seen. It's not as common with Ambras syndrome.

Acquired Ambras syndrome can be a sign of cancer. Localized cases of hypertrichosis can be genetic as well as acquired. When it's acquired it's generally due to medications or physical trauma.

burcidi
Post 2

Is Ambras syndrome the same as the hypertrichosis syndrome? Hypertrichosis is also an abnormal growth of hair on face and body and is caused by a genetic mutation. It sounds the same as the Ambras syndrome.

I had a long discussion about this syndrome in a forum once. There I learned that some scientists actually claim that Ambras syndrome is not a genetic mutation but rather gene expression. They claim that this is a gene that existed in our primal ancestors but was turned off during our evolution to our present state.

I don't know what kind of research these scientists have done to back up this claim, but personally this sounds pretty logical. The reason I believe

this is because most people who have been documented to have Ambras and Hypertrichosis syndrome throughout history didn't have any other abnormal features or any health problems aside from having excessive hair. (The dental condition mentioned in this article is probably a part of this gene expression).

There are several individuals in the world right now that have Ambras syndrome and doctors have found them to be completely healthy. So maybe this really is a condition where the turned off gene has simply been turned on.

What do you think?

ddljohn
Post 1

There is a little girl in Thailand who has this condition. It was on the news a couple of times because she was given the Guinness Wold Record for being the world's most hairiest girl. She was so happy about it because she said it made her more popular at school.

They also talked about how she had a breathing problem when she was born and was hospitalized for a long time. Her family had her get laser treatments when she was a little older to remove the hair but apparently it didn't help at all. Her hair which covers her face entirely as well as some areas on her body is getting longer and thicker every year.

She was putting up a very courageous and confident attitude in her interviews and I really admire her for that. I'm sure life is not going to be very easy for her as people are just not knowledgeable about this genetic condition and will stare at her.

I really hope that a treatment will be found for her body and face hair though. That appears to be the only issue she has now since her breathing issues were fixed after birth with surgery.

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