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Behcet Disease
(Adamantiales-Behcet Syndrome)
Definition
Behcet disease is a rare, chronic disorder. Blood vessels are inflamed throughout the body. It causes ulcers in the mouth and genitals, and inflammation in the eyes.
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’s not known what causes this problem. It may be related to genetics or a reaction to an infection.
Risk Factors
Your chances of Behcet disease are higher if you:
- Are aged 20-39 years old
- Are male in the US
- Are female in the Middle East, Asia, or Japan
- Have a family history
- Carry 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:
- Affect almost all people with the disease
- Usually are the first to appear
-
Ulcerated Gums Copyright © Nucleus Medical Media, Inc. - Genital sores
-
Eye disease:
- Redness
- Blurry vision
- Light sensitivity
- Watery eyes
- Joint swelling and pain—usually does not cause permanent joint damage
-
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
Serious problems associated with Behcet:
- Excess tiredness
- Blood clots—especially in the legs
- Aneurysm
-
Heart problems such as:
- Abnormal heart rhythms
- Inflammation of the heart muscle— myocarditis
-
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 Copyright © Nucleus Medical Media, Inc. -
Digestive problems:
- Causes ulcers and inflammation of the digestive tract—rare
These problems need prompt medical care.
Diagnosis
Diagnosing Behcet disease is difficult because symptoms:
- Are similar to those of many other diseases
- Often appear slowly, over months or years
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
You may have a pathergy skin test. The skin is pricked with a small needle. If you have Behcet disease, a bump will develop at the site of the skin prick. However, this test is not conclusive. 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.
Medicines
Topical medicines include creams, lotions, and mouth rinses. They help ease swelling and pain.
Other medicines are taken by mouth to:
- Ease swelling and pain
- To change the response of the immune system
You and your doctort will watch for serious side effects to these medicines.
Rest and Exercise
General guidelines include:
- Rest when symptoms flare to help speed healing.
- Engage in moderate exercise when symptoms recede to help keep joints flexible and strong.
RESOURCES
American Behcet's Disease Association
http://www.behcets.com
http://www.behcets.com
National Institute of Arthritis and Musculoskeletal and Skin Diseases
https://www.niams.nih.gov
https://www.niams.nih.gov
CANADIAN RESOURCES
Arthritis Society
http://www.arthritis.ca
http://www.arthritis.ca
Health Canada
https://www.canada.ca
https://www.canada.ca
References
Behçet disease. Genetics Home Reference website. Available at: https://ghr.nlm.nih.gov/condition/behcet-disease. Updated July 3, 2018. Accessed July 9, 2018.
Behçet disease. Merck Manual Professional Version website. Available at: https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/beh%C3%A7et-disease. Updated September 2017. Accessed July 9, 2018.
Behcet syndrome. EBSCO DynaMed Plus website. Available at:
http://www.dynamed.com/topics/dmp~AN~T114300/Behcet-syndrome
. Updated April 2, 2018. Accessed July 9, 2018.
Behcet's disease. American Behcet's Disease Association website. Available at: http://www.behcets.com/site/c.8oIJJRPsGcISF/b.9196317/k.904C/Behcets%5FDisease.htm. Accessed July 9, 2018.
Revision Information
- Reviewer: EBSCO Medical Review Board Michael J. Fucci, DO, FACC
- Review Date: 05/2018
- Update Date: 07/09/2018