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BEHCET’S SYNDROME

Nervous System Diseases, Skin Conditions / By

BEHCET’S SYNDROME

Behcet’s syndrome is a rare inflammatory disorder that damages the body’s blood vessel.

  • The condition is not contagious and cannot spread from person to person. However, multiple parts of the body get affected.
  • It is a chronic condition and severity varies from person to person.
  • Onset is usually in people in their 20s and 40s and affects both men and women
  • While common in Turkey, the Middle East, and Asia, it is less common in the United States and Europe.
  • The American Behcet’s Disease Associations provide that the prevalence of the disease in Turkey is as high as 400 cases per 100,000 people.
  • Symptoms may temporarily disappear and reoccur at a later time.

CAUSES

The exact cause of the condition is unknown. Because the symptoms are related to inflammation of the blood vessels, it may be that a person inherits an immune system disorder that affects their arteries and veins.

Some researchers believe that a virus or bacterium may trigger Behcet’s syndrome in people with certain genes making them more susceptible.

People with one kind of autoimmune disorder such as rheumatoid arthritis may be at greater risk of getting the condition.

People who are from countries in the Middle East and the Far East including Turkey, Iran, Japan, and China are more likely to develop the condition.

SYMPTOMS

Signs and symptoms may depend on the area of the body affected. Areas commonly affected by the condition include:

  • Mouth – painful sores in the mouth that may look similar to a canker sore. They may be raised, round, deep lesions in the mouth that turns into painful ulcers. These sores may be on the tongue, lips, gums, a roof of the mouth, or the back of the throat. These sores often heal within 10 to 20 days
  • Eyes – the condition can cause inflammation of the middle layer in the eyes. This condition is called uveitis. Uveitis may affect the front of the eye (anterior) and the back of the eye and retina (posterior). Uveitis may be characterized by blurry vision, redness, pain, and sensitivity to light. If left untreated, eye problem caused by Behcet’s syndrome may cause partial or total blindness.
  • Skin – skin problem may vary. While some people develop acne-like sores some people may develop raised and tender nodules that are often ulcerated on the skin especially on the lower legs.
  • Genitals – over 50% of those with the condition will develop genitals lesions. The sores are painful, red, and open and are commonly on the scrotum or vulva.
  • Joints – joint swelling and pain may occur in the knees, elbows, ankles, and wrists. This can last for two to three weeks and resolve on their own.
  • Brain- the condition can also lead to inflammation of the brain and the brain stem. This can lead to headache, fever, poor balance, memory loss, disorientation, and stroke.
  • Vascular systems – inflammation of the arteries and veins may occur in Behcet’s syndrome and can lead to serious complications such as aneurysm and blocked or narrowed vessels.
  • Digestive systems – variety of symptoms including abdominal pain, bleeding and diarrhea may occur in the digestive system.

DIAGNOSIS AND TREATMENT

There is no test that can determine definitively the presence of Behcet’s syndrome. The doctors may have to rely primarily on your signs and symptoms to make diagnosis.

A mouth sore that have recurred at least three times in a year plus any two of the following may be used as criteria for diagnosis;

  • Genital sores that appear and then disappear
  • Skin sores
  • Eye inflammation that affects vision. This can be identified by an ophthalmologist
  • Positive pathergy test where a sterile needle is inserted into your skin and then examined. A small red bump under the skin may indicate that the pathergy test is positive; this means your immune system is overreacting to stimulus.
TREATMENT

Treatment may depend on the severity

For mild cases, anti-inflammatory medications such as ibuprofen may be used to control temporary flares in pain and inflammation.

Topical corticosteroids may be applied to the skin and genital in order to reduce pain and inflammation

Special mouthwashes that contain corticosteroids, as well as eye drops containing corticosteroids, may be used to reduce pain in the mouth and eye respectively.

If topical medications don’t work, a strong anti-inflammatory drug called colchicine may be prescribed in severe cases.

Corticosteroids such as prednisone may be used to reduce inflammation in severe cases

Immunosuppressive drugs such as azathioprine, cyclosporine may be prescribed, this helps to keep your immune system from attacking healthy tissues

Medications such as interferon alfa-2b may be used to control inflammation. This medication regulates the activity of the immune system