What is Behçet's disease?
Behçet's disease or syndrome is a chronic (long term)
inflammatory condition caused by vasculitis (an inflammation of the blood
vessels) that results in damage to both arteries and veins. The cause of
Behçet's disease is unknown.
Who gets Behçet's disease?
Behçet's disease is seen worldwide. It is most common
in the Mediterranean basin and Middle East (up to 300 cases/100,000 population)
and Far East (about 15 cases/100,000 population). In the US, it is less common
(about 7 cases/100,000 population). Men and women are affected with equal
frequencies. The disease can develop at any age but most often begins between ages 20-30 years.
What are the symptoms of Behçet's disease?
Recurrent sores in the mouth and/or genital area,
skin, joint pain, and inflammation in the eyes are the key symptoms of Behçet’s
disease. The brain, nerves, lungs (rare), intestinal tract and kidneys can also be involved.
Where the disease strikes in the body varies from person to person. Some of these sites and key features include:
- Mouth sores occur at some time in all patients. They are usually
recurrent and painful and affect almost all patients with Behçet's disease.
The sores look like the common canker sore, but are more numerous, frequent
and painful. They are often the first symptom that a person notices and may
occur long before any other symptoms appear. Mouth ulcers can be found on
the lips, tongue, and inside the cheek.
- Genital sores look similar to mouth sores and may be painful, but
they are not as common as mouth sores. They can be on the scrotum (the sack
enclosing the testes) in the male and on the vulva (the external genital
organs) in the female.
- Eye inflammation can result in pain, blurry vision, light
sensitivity, tearing, or redness of the eye. Behçet’s may even lead to
vision loss. Severe eye disease that can cause blindness is more common in
the Middle East and Japan than in the US.
- Skin problems are a common symptom of Behçet's disease. They may
look like acne, or have other appearances such as coin-shaped nodules that
are tender (called erythema nodosum) or ulcers that may be either
superficial or deep and are painful. Patients with Behçet's disease may
develop a red bump or sore when the skin is scratched or pricked. Doctors
refer to this event as a positive pathergy test.
- Joint pain is common. The most frequently affected sites are the
ankles, knees, elbows and hips. Inflammation in the joint can cause
swelling, redness and tenderness, but usually does not lead to permanent damage.
- Veins. Inflammation of veins may lead to the development of clots at
those sites and the blocking or closing of those vessels. This may affect
superficial veins in the skin or deep veins, including the largest in the
body (called the vena cava), where the consequences to health are more
serious. This is a consequence of inflammation and not a flaw in the clotting system.
- Brain. The brain can be affected, most often the meninges (the brain
covering). Inflammation may be associated with fever, headache, stiff neck,
and difficulty coordinating movement. Brain involvement can also include
stroke, which is due to blockage or rupture of blood vessels in the brain.
- Gastrointestinal (GI) tract features include abdominal pain or blood
in the stools, which results from lesions similar to those seen in the mouth and genital area. These lesions can be more dangerous
in the GI tract because they are likely to bleed and/or rupture the intestine.
- Other organs like the lungs, kidney, and large vessels (such as the aorta) can occasionally be involved.