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by Scholten A

Behcet Disease

(Adamantiales-Behcet Syndrome)

Definition

Behcet disease is a rare, long-term disorder. Blood vessels are inflamed throughout the body. It causes ulcers in the mouth and genitals, and inflammation in the eyes. Treatment improves outcomes.

Causes

The cause of Behcet disease is unknown. The body’s immune system may be too active. When this happens, healthy tissue is attacked. It may be due to genes or a reaction to an infection.

Risk Factors

Behcet disease is more common in men from the Middle East and Mediterranean. It is also more common people aged 20 to 39 years old. Other things that raise the risk are:
  • A family history of Behcet disease
  • Having a certain gene

Symptoms

Symptoms vary from mild to very serious. They also tend to come and go over months or years. The most common are:
  • Sores in the mouth:
  • Ulcerated Gums
    Periodontal Disease
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  • Genital sores
  • Eye problems, such as:
    • Redness
    • Blurry vision
    • Light sensitivity
    • Watery eyes
  • Joint swelling and pain
  • Skin problems such as sores that:
    • May appear as red, raised sores or bumps, or may be flat
    • Usually appear on the legs and upper part of the body
  • Excessive tiredness
Behcet can also cause serious problems such as:
  • Blood clots—especially in the legs
  • Aneurysm
  • Heart problems such as:
  • Central nervous system problems such as:
    • Inflammation of the brain and membrane that lines the brain
    • Headache
    • Problems with moving around or speaking
  • Stroke from a Cerebral Aneurysm
    Cerebral Aneurysm
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  • Digestive problems:
    • Causes ulcers and inflammation of the digestive tract—rare
These problems need medical care right away.

Diagnosis

Diagnosing Behcet disease is difficult. Symptoms appear slowly, over months or years. They are similar to symptoms of many other diseases.
There are no specific tests to diagnose Behcet disease. It may be suspected based on:
Mouth sores at least 3 times within a year and at least 2 of these:
  • Eye inflammation
  • Genital sores
  • Skin sores
A pathergy skin test may be used. The skin is pricked with a small needle. If a bump develops at the site of the skin prick, it could mean Behcet disease. However, many people with the disease do not have a reaction to the skin prick.

Treatment

For some people, the illness may go away on its own. If care is needed, it focuses on treating problems to prevent serious complications. With proper care, Behcet disease can be managed.
Treatment options may be:
  • Creams, lotions, and mouth rinses—to help ease swelling and pain
  • Other medicines taken by mouth to:
    • Ease swelling and pain
    • Change how the immune system responds
  • Lifestyle changes such as:
    • Resting when symptoms flare
    • Exercising to keep joints flexible and strong

Prevention

There are no guidelines to prevent Behcet disease.

RESOURCES

American Behcet's Disease Association
http://www.behcets.com
National Institute of Arthritis and Musculoskeletal and Skin Diseases
https://www.niams.nih.gov

CANADIAN RESOURCES

Arthritis Society
http://www.arthritis.ca
Health Canada
https://www.canada.ca

References

Behcet's disease. American Behcet's Disease Association website. Available at: https://www.behcets.com/basics-of-behcets/. Accessed July 16, 2021.
Behçet disease. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/beh%C3%A7et-disease. Accessed July 16, 2021.
Behcet syndrome. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/behcet-syndrome. Accessed July 16, 2021.
Davatchi F. Behçet's disease. Int J Rheum Dis. 2018;21(12):2057-2058.

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