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by Alan R

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

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

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.

Prevention

Behcet disease can’t be prevented because the cause is unknown.

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

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

EBSCO Information Services is fully accredited by URAC. URAC is an independent, nonprofit health care accrediting organization dedicated to promoting health care quality through accreditation, certification and commendation.

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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