Behcet Syndrome

What is Behcet Syndrome

Behcet syndrome is a long-term (chronic) condition that causes swelling and irritation (inflammation) of blood vessels (vasculitis). This inflammation can lead to painful mouth ulcers (canker sores) and skin ulcers.

Canker sores may appear anywhere in the mouth. Behcet syndrome may also affect other parts of the body, including the eyes, joints, and genitals.

Behcet syndrome may be passed down to family members through an abnormal gene.

This gene may cause your immune system to mistakenly attack blood vessels, causing inflammation (autoimmune disorder). In other cases, the disorder may occur without a family history.

What are the causes?

The exact cause of this condition is not known.

What increases the risk?

This condition is more likely to occur in people who:

  • Have a parent who carries the gene that is associated with the condition. The gene is more common in people of Middle Eastern and Asian descent.
  • Are between 20 and 40 years old.
  • Are male.

What are the signs or symptoms?

Symptoms of this condition may come and go and can range from mild to severe. Common symptoms include:

  • Canker sores.
  • Genital ulcers.
  • Skin ulcers.
  • Painful skin bumps or acne.
  • Eye inflammation, which can cause eye pain, redness, tearing, and blurred vision.
  • Joint pain and inflammation (arthritis).

Less common symptoms include:

  • Digestive system inflammation, which can cause nausea, belly pain, diarrhea, or bloody diarrhea.
  • Brain inflammation, which can cause headaches and clumsiness.
  • Large blood vessel inflammation, which can cause blood clots.
  • Lung or heart inflammation, which can cause breathing difficulty or chest pain.

How is this diagnosed?

This condition is diagnosed based on your symptoms, medical history, and a physical exam. Your health care provider may also do a skin test to confirm the diagnosis. This involves pricking the skin with a needle to see if small red bumps form (pathergy test).

How is this treated?

There is no cure for this condition. Treatment depends on your symptoms. Treatment may include steroid medicines, such as:

  • Steroid creams or ointments for oral, genital, or other skin ulcers.
  • Numbing steroid mouthwash for painful mouth ulcers.
  • Steroid eye drops for eye symptoms.
  • Oral steroid medicine to treat a flare-up of symptoms.

Other medicines used to treat symptoms of this condition may include:

  • NSAIDs to relieve pain and swelling.
  • Medicines that suppress the immune system response for severe symptoms (immunosuppressive medicines).
  • A medicine for severe symptoms that do not respond to other treatments (TNF inhibitor).

Follow these instructions at home:

  • Learn as much as you can about your condition.
  • Work closely with your team of health care providers.
  • Take or apply over-the-counter and prescription medicines only as told by your health care providers.
  • Rest at home during a flare-up of symptoms.
  • Return to your normal activities as told by your health care providers. Ask your health care providers what activities are safe.
  • Keep all follow-up visits as told by your health care provider. This is important.

Where to find more information

Contact a health care provider if:

  • You have a fever.
  • You have a flare-up of Behcet symptoms.
  • You have side effects from medicines.
  • You develop any new symptoms of Behcet syndrome.

Get help right away if:

  • You have a severe headache.
  • You become unusually clumsy.
  • You have a sudden change in vision.
  • You have chest pain or difficulty breathing.
  • You vomit blood or have bloody stool.

Summary

  • Behcet syndrome is a long-term (chronic) condition that causes swelling and irritation (inflammation) of blood vessels (vasculitis).
  • Treatment may include steroid medicines and other medicines to relieve pain and swelling.
  • During symptom flare-ups, rest at home and then return to your normal activities as told by your health care provider.

Behcet Syndrome, Pediatric

Behcet syndrome is a long-term (chronic) condition that causes swelling and irritation (inflammation) of blood vessels (vasculitis). This inflammation can lead to painful mouth ulcers (canker sores) and skin ulcers. Canker sores may appear anywhere in the mouth. Behcet syndrome may also affect other parts of the body, including the eyes, joints, and genitals.

Behcet syndrome may be passed down to family members (inherited) through an abnormal gene. This gene may cause your child’s immune system to mistakenly attack blood vessels, causing inflammation (autoimmune disorder). In some cases, the disorder may occur without a family history.

What are the causes?

The exact cause of this condition is not known.

What increases the risk?

Your child may be at higher risk for Behcet syndrome if:

  • A parent carries the gene for the condition. This gene is more common in people of Middle Eastern and Asian descent.

What are the signs or symptoms?

Symptoms of this condition may come and go and can range from mild to severe. Canker sores are the most common symptom in children. Other symptoms include:

  • Genital ulcers.
  • Skin ulcers.
  • Painful skin bumps or acne.
  • Eye inflammation, which can cause eye pain, redness, tearing, and blurred vision.
  • Joint pain and inflammation (arthritis).

Less common symptoms include:

  • Digestive system inflammation, which can cause nausea, abdominal pain, diarrhea, or bloody diarrhea.
  • Brain inflammation, which can cause headaches and clumsiness.
  • Large blood vessel inflammation, which can cause blood clots.
  • Lung or heart inflammation, which can cause breathing difficulty or chest pain.

How is this diagnosed?

This condition is diagnosed based on your child’s symptoms, medical history, and a physical exam. Your child’s health care provider may also do a skin test to confirm the diagnosis. This involves pricking the skin with a needle to see if small red bumps form (pathergy test).

How is this treated?

There is no cure for Behcet syndrome. Treatment depends on your child’s symptoms. Treatment may include steroid medicines, such as:

  • Steroid creams or ointments for oral, genital, or other skin ulcers.
  • Numbing steroid mouthwash for painful mouth ulcers.
  • Steroid eye drops for eye symptoms.
  • Oral steroid medicine to treat a flare-up of symptoms.

Other medicines used to treat symptoms of Behcet syndrome may include:

  • NSAIDs to relieve pain and swelling.
  • Medicines that suppress the immune system response (immunosuppressive drugs) for severe symptoms.
  • A medicine for severe symptoms that do not respond to other treatments (TNF inhibitor).

Follow these instructions at home:

  • Learn as much as you can about your child’s condition.
  • Work closely with your child’s team of health care providers and take an active role in treatment.
  • Give or apply over-the-counter and prescription medicines only as told by your child’s health care providers.
  • Have your child rest at home during a flare-up of symptoms.
  • Have your child return to his or her normal activities as told by the health care providers. Ask your child’s health care providers what activities are safe for your child.
  • Keep all follow-up visits as told by your child’s health care providers. This is important.

Where to find more information

Contact a health care provider if:

  • Your child has a fever.
  • Your child has a flare-up of Behcet symptoms.
  • Your child has side effects from medicines.
  • Your child develops any new symptoms of Behcet syndrome.

Get help right away if:

  • Your child has a severe headache.
  • Your child becomes unusually clumsy.
  • Your child has a sudden change in vision.
  • Your child has chest pain or difficulty breathing.
  • Your child vomits blood or has bloody stool.
  • Your child who is younger than 3 months has a temperature of 100°F (38°C) or higher.

Summary

  • Behcet syndrome is a chronic condition that causes inflammation of blood vessels (vasculitis).
  • The exact cause of this condition is not known.
  • Your child may be at higher risk for Behcet syndrome if a parent carries the gene for this condition.
  • Symptoms of this condition may come and go and can range from mild to severe.
  • There is no cure for Behcet syndrome. Treatment depends on your child’s symptoms. Work closely with your child’s team of health care providers and take an active role in treatment.
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