Behcet's Disease


Fact: Behcet’s disease is common in the Middle East, Asia, and Japan;
​it is rare in the United States

What is it?
Behcet's Disease, also known as Behcet's syndrome, is a rare, chronic, autoimmune, autoinflammatory disorder of unknown origin. Its manifestations are thought to be caused by vasculitis resulting in damage to blood vessels throughout the body. The disease is named for the Turkish dermatologist, Dr. Hulusi Behcet, who in 1937, described a triad of oral ulcers, genital ulcers and ocular inflammation. Although Behcet's Disease is recognized worldwide, prevalence is highest in countries in the eastern Mediterranean, the Middle East, and East Asia, along the ancient Silk Road.

Symptoms
Behcet's disease symptoms vary from person to person. It may disappear and recur on its own. Symptoms may become less severe over time. Your signs and symptoms depend on which parts of your body are affected.

​Areas commonly affected by Behcet's disease include:

  • Mouth. Painful mouth sores that look similar to canker sores are the most common sign of Behcet's disease. They begin as raised, round lesions in the mouth that quickly turn into painful ulcers. The sores usually heal in one to three weeks, though they do recur.
  • Skin. Skin problems can vary. Some people may develop acne-like sores on their bodies. Others may develop red, raised and tender nodules on their skin, especially on the lower legs.
  • Genitals. People with Behcet's disease may develop sores on their genitals. The red, open sores commonly occur on the scrotum or the vulva. The sores are usually painful and may leave scars.
  • Eyes. Behcet's disease may cause inflammation in the eye — a condition called uveitis (u-vee-I-tis). Uveitis causes redness, pain and blurred vision in one or both eyes. In people with Behcet's disease, the condition and may come and go.
  • Joints. Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also may be involved. Signs and symptoms may last one to three weeks and go away on their own.
  • Vascular system. Inflammation in blood vessels (veins and arteries) may occur in Behcet's disease, causing redness, pain, and swelling in the arms or legs when a blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.
  • Digestive system. Behcet's disease may cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea and bleeding.
  • Brain. Behcet's disease may cause inflammation in the brain and nervous system that leads to headache, fever, disorientation, poor balance or stroke.

Make an appointment with your doctor if you notice any unusual signs and symptoms that might indicate Behcet's disease

Causes
No one knows exactly what causes Behcet's disease. But it may be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells. It's likely that genetic and environmental factors also play a role. 

Several genes have been found to be associated with the disease. Some researchers believe a virus or bacterium may trigger Behcet's disease in people who have certain genes that make them susceptible to Behcet's.

Risk factors
Factors that may increase your risk of Behcet's include:

  • Age. Behcet's disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition.
  • Where you live. People from countries in the Middle East and Far East, including Turkey, Iran, Japan and China, are more likely to develop Behcet's.
  • Sex. While Behcet's disease occurs in both men and women, the disease is usually more severe in men.
  • Genes. Having certain genes is associated with a higher risk of developing Behcet's.


Complications
Complications of Behcet's disease depend on your signs and symptoms. For instance, untreated uveitis can lead to decreased vision or even blindness.

People with eye signs and symptoms of Behcet's disease need to visit an eye doctor (ophthalmologist) regularly because treatment can help prevent this complication.

How Is Behcet’s Disease Diagnosed?
Diagnosing Behcet’s disease is very difficult because no specific test confirms it. Less than half of people initially thought to have Behcet’s disease actually have it. When a patient reports symptoms, the doctor must conduct an examination and rule out other conditions with similar symptoms.

Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time. A patient may even visit several different kinds of doctors before the diagnosis is made.

These symptoms are key to a diagnosis of Behcet’s disease:

  • Mouth sores at least three times in 12 months.
  • Any two of the following symptoms: recurring genital sores, eye inflammation with loss of vision, characteristic skin lesions, or positive pathergy (skin prick test).


​Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Crohn’s disease and reactive arthritis. The doctor also may recommend that the patient see an eye specialist to identify possible complications related to eye inflammation. A dermatologist may perform a biopsy of mouth, genital, or skin lesions to help distinguish Behcet’s from other disorders

Treatment
No cure exists for Behcet's disease. If you have a mild form of the condition, your doctor may offer medications to control temporary flares in pain and inflammation. You may not need to take medication between flares.

If your signs and symptoms are more severe, your doctor may advise medications to control the signs and symptoms of Behcet's disease throughout your body, in addition to medications for the temporary flares.

Treatments for individual signs and symptoms of Behcet's diseaseYour doctor works to control any signs and symptoms you experience during flares with medications such as:

  • Skin creams, gels and ointments. Topical corticosteroid medicines are applied directly to skin and genital sores in order to reduce inflammation and pain.
  • Mouth rinses. Special mouthwashes that contain corticosteroids and other agents to reduce the pain of mouth sores may ease your discomfort.
  • Eyedrops. Eyedrops containing corticosteroids or other anti-inflammatory medicines can relieve pain and redness in your eyes if inflammation is mild.


Systemic treatments for Behcet's disease
If topical medications don't help, your doctor may recommend a drug called colchicine (Colcrys). Arthritis symptoms also may improve with colchicine.

Severe cases of Behcet's disease require treatments to control damage from the disease between flares. If you have moderate to severe Behcet's disease, your doctor may prescribe:

  • Corticosteroids to control inflammation. 
    Corticosteroids, such as prednisone, may reduce the inflammation caused by Behcet's disease. The signs and symptoms of Behcet's disease tend to recur when corticosteroids are used alone, so doctors often prescribe them with another medication to suppress the activity of your immune system (immunosuppressives).
    Side effects of corticosteroids include weight gain, persistent heartburn, high blood pressure and bone thinning (osteoporosis).
  • Medications that suppress your immune system. 
    By stopping your immune system from attacking healthy tissues, immunosuppressive drugs reduce the inflammation. Immunosuppressive drugs that may play a role in controlling Behcet's disease include azathioprine (Imuran, Azasan), cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide (Cytoxan).
    Because these medications suppress the actions of your immune system, they may increase your risk of infection. Other possible side effects include liver and kidney problems, low blood counts, and high blood pressure.
  • Medications that alter your immune system's response. 
    Interferon alfa-2b (Intron A) regulates the activity of your immune system to control inflammation. It may be used alone or with other drugs to help control skin sores, joint pain and eye inflammation in people with Behcet's disease.


Side effects include flu-like signs and symptoms, such as muscle pain and fatigue.

Medications that block a substance called tumor necrosis factor (TNF) are effective in treating some of the signs and symptoms of Behcet's, especially for people who have more-severe or resistant symptoms. Examples include infliximab (Remicade) and etanercept (Enbrel).

Side effects may include headache, skin rash and an increased risk of upper respiratory infections.

Want to learn more?
The following are organizations and/or websites dedicated to providing information and education surrounding Behcet's Disease. These organizations are dedicated to research, education, awareness, and/or support. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.
American Behcet's Disease Association

NIH
​Mayo Clinic
WebMD

Information on this page taken directly from:
​​American Behcet's Disease Association
NIH
​Mayo Clinic