Behcet’s Syndrome

What Is Behcet’s Syndrome?

Behcet’s syndrome (also called Behcet’s disease) is rare condition that causes your body’s blood vessels to swell. This can cause symptoms throughout your body. Sores inside your mouth and on your genitals are common, but you may also have symptoms in other areas.

There’s no cure for Behcet’s syndrome, but there are ways to treat the symptoms.

Behcet’s Syndrome Signs and Symptoms

Behcet’s syndrome is different for everyone. Some people have only mild symptoms. Others have more serious problems.

Your symptoms often come and go. Bouts of symptoms are called flares. Going long periods without symptoms is called remission.

Most people with Behcet’s syndrome have sores in the mouth and on the genitals. But the swelling of blood vessels it causes can bring on symptoms throughout your body:

Mouth:

  • Sores on the lips, tongue, and inside of the cheek
  • Mouth pain

Eyes:

Genitals:

  • Sores on the scrotum in men
  • Sores on the vulva in women

Skin:

  • Bumps that look like acne
  • Red lumps on the legs and ankles

Joints:

Brain and spinal cord:

Digestive system:

Sometimes, Behcet’s affects other organs, like the kidneys and lungs.

Behcet’s Syndrome Causes and Risk Factors

Doctors aren’t sure what causes Behcet’s syndrome. They believe it starts when the immune system mistakenly attacks the blood vessels.

You’re more likely to get it if you have a problem with your immune system. Some people inherit genes that cause their immune system to attack the blood vessels. Doctors believe an infection or something else in the environment triggers the condition in those who have the gene.

Risk factors

Some things can make you more likely to have Behcet’s syndrome, including:

  • Age: Although you can get it at any age, it usually shows up when you’re in your 20s or 30s.
  • Genes: Certain genes (a portion of your DNA) are linked to this condition.
  • Sex: Although anyone can get it, it affects more men, more severely, than women.
  • Where you live: Behcet’s syndrome is very rare in the U.S. It’s more common in the Middle East and Asia.

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Behcet’s Syndrome Diagnosis

Because there are so many different symptoms, Behcet’s syndrome can be hard to diagnose. It has symptoms similar to many other conditions, including Crohn’s disease, lupus, and arthritis. Your doctor may want to give you a blood test to rule out other conditions. It can take months to find out for sure that you have Behcet’s.

Doctors will look at your medical history, do a physical exam, and look for groups of symptoms. You may be diagnosed with Behcet’s if you have mouth sores at least three times in a year, and you have at least two of these symptoms:

  • Sores on your genitals
  • Swelling in your eyes, along with vision loss
  • Skin sores
  • Positive pathergy test: Your doctor will prick your arm with a small needle. If a bump forms in that spot 1 or 2 days later, it’s a good clue that you may have the condition. It indicates an overreaction by your immune system to a small injury.

Doctors who treat Behcet’s

Which doctors you see will depend on your symptoms:

Because you may have many symptoms, you may need to visit several of these doctors.

Behcet’s Syndrome Treatment

Your treatments will be based on your symptoms.

Corticosteroids can calm the immune system and bring down swelling in your body.

Other medicines that work to help your immune system response can also be prescribed, such as adalimumab (Humira), azathioprine (Imuran), cyclophosphamide, cyclosporine (Neoral, Sandimmune), and infliximab (Remicade).

Creams or ointments can ease skin and genital sores.

Your doctor may recommend nonsteroidal anti-inflammatory drugs, or NSAIDs, and colchicine for joint pain and swelling.

Steroid eye drops can bring down eye swelling.

Behcet’s Syndrome Complications

Depending on your symptoms, it’s possible you could get complications from Behcet’s syndrome. But not everyone will. It’s important to see your doctor if you are noticing new symptoms. For example, if you have eye inflammation that goes untreated, it can result in loss of vision. Treatment can help prevent this.

Blood clots can also cause serious complications. A pulmonary artery aneurism (when there’s a bulge in your artery that can burst) is rare but can be fatal. Inflammation in your brain or spinal cord can also cause disability. Always call your doctor if you have questions or new symptoms.

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Behcet’s Syndrome Outlook

You can live an active life with Behcet’s. Your symptoms might get better as you grow older.

There may be periods of time when all symptoms disappear.

For some, the symptoms get worse over time. Medicines help control these symptoms and prevent flares.

WebMD Medical Reference Reviewed by Stephanie S. Gardner, MD on July 27, 2020

Sources

SOURCES:

American Behcet’s Disease Association: “Diagnosis.”

Cleveland Clinic: “Behcet’s Disease.”

Genetics Home Reference: “Behcet Disease.”

Johns Hopkins: “Behcet’s Disease.”

Mayo Clinic: “Behcet’s Disease.”

National Eye Institute: “Facts About Behcet’s Disease of the Eye.”

National Institute of Arthritis and Musculoskeletal and Skin Diseases: “Questions and Answers about Behcet��s Disease.”

National Institute of Neurological Disorders and Stroke: “What is Behcet’s Disease?”

National Organization for Rare Disorders: “Behcet’s syndrome.”

Vasculitis Foundation: “Behcet’s Disease.”

Brigham and Women’s Hospital: “Behcet’s Disease.”

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