Definition

Behcets disease is a rare, chronic disorder involving inflammation of blood vessels throughout the body. It is marked by recurrent oral and genital ulcers and eye inflammation.

Causes

The cause of Behcets disease is unknown. It is believed to be caused by an autoimmune reaction. This occurs when the body’s immune system attacks its own tissue by mistake. This autoimmune abnormality may be inherited. An environmental trigger, such as a specific virus or bacterium, may activate the disease in people susceptible to it.

Risk Factors

Although the exact cause of Behcets disease is unknown, some groups of people are more likely to develop the condition than others. Behcets disease is more common in people who are 20-39. In the United States, men are more likely than women to develop Behcets. In the Middle East, Asia, and Japan, women are more likely than men to develop Behcets.

Factors that may increase your risk of Behcets include:

• Family history of Behcets disease
• A gene marker called the HLA-B51/B5 allele

Symptoms

Symptoms of Behcets disease can vary from mild to very severe. Symptoms tend to appear, heal, and then recur (referred to as a flare) frequently over months or years. The most common symptoms of the disease are:

• Oral sores
  • Affect almost all people with the disease
  • Usually are the first to appear
  • Usually last for 10-14 days
  • Can be painful
  • Can cause scarring

Ulcerated Gums Copyright © Nucleus Medical Media, Inc.

• Genital sores
  • In men: appear on the penis and/or scrotum
  • In women: appear on the vulva or within the vagina
  • Can be painful
  • Can cause scarring
• Uveitis—inflammation of the middle part of the eye, including the iris
  • Tends to cause eye redness, blurred vision, sensitivity to light, and watering eyes
  • If not treated, can cause partial vision loss or blindness
• Arthritis —inflammation of joints
  • Tends to be painful
  • Usually does not cause permanent joint damage
• Skin problems, such as sores
  • May appear as red, raised sores or bumps, or may be flat (flush with the skin)
  • Usually appear on the legs and upper torso

Complications of the disease may include:

• Severe fatigue during a flare
• Blood clots
  • Caused by thrombophlebitis (inflammation of veins), usually in the legs
  • Can cause severe complications if not treated quickly
• Aneurysms, or severely dilated blood vessels
  • May rupture and cause severe consequences
• Heart problems, such as:
  • Abnormal heart rhythms
  • Missed heartbeats
  • Early heartbeats
  • Inflammation of the heart muscle ( myocarditis)
• Central nervous system problems
  • For example, meningoencephalitis, which is inflammation of the brain and membrane that lines the brain
  • May result in seizures, confusion, strokes, memory problems, headaches

Stroke from a Cerebral Aneurysm Copyright © Nucleus Medical Media, Inc.

• Digestive problems
  • Causes ulcers and inflammation of the digestive tract (rarely)

Diagnosis

Diagnosing Behcets disease is very difficult because:

• Symptoms are similar to those of many other diseases.
• Symptoms often appear slowly, over months or years.
• There is no specific test to confirm the disease.

Behcets disease may be suspected if oral sores appear at least 3 times within a year and at least 2 of the following recurring symptoms appear:

• Eye inflammation
• Genital sores
• Skin sores

If Behcets disease is suspected, you may have a pathergy skin test. For this test, your skin is pricked with a small needle. If you have Behcets 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

There is no cure for Behcets disease however, spontaneous regression may occur. Treatment is aimed at limiting and preventing complications from symptoms. With proper treatment, the disease can usually be managed fairly well. Treatment includes medications, rest, and exercise.

Prevention

There are no guidelines for the prevention of Behcets disease because the exact cause is unknown.

National Institute of Arthritis and Musculoskeletal and Skin Diseases http://www.niams.nih.gov

Health Canada http://www.hc-sc.gc.ca

Behçet disease. Genetics Home Reference website. Available at:
http://ghr.nlm.nih.gov/condition/behcet-disease
Updated July 2010. Accessed June 30, 2015.

Cakir O, Eren N, Ulka R, Nazaroghi H. Bilateral subclavian arterial aneurysm and ruptured abdominal aorta pseudoaneurysm in Behcet’s disease. Ann Vasc Surg. 2002,16:516-520

Okada AA. Behcet’s disease: general concepts and recent advances. Curr Opin Ophthalmol. 2006;17:551-556.