What is Behcet's Disease?

Behcet’s Disease Overview

Behcet’s (beh-CHETS) disease, also called Behcet’s syndrome, is a rare disorder that causes inflammation in blood vessels throughout your body. The inflammation of Behcet’s disease leads to numerous symptoms that may initially seem unrelated. The signs and symptoms of Behcet’s disease which may include mouth sores, eye inflammation, skin rashes and lesions, and genital sores vary from person to person and may come and go on their own.

The exact cause of Behcet’s is unknown, but it may be an autoimmune disorder, which means the body’s immune system mistakenly attacks some of its own healthy cells. Both genetic and environmental factors may be responsible for Behcet’s disease.

Treatment aims to reduce the signs and symptoms of Behcet’s disease and to prevent serious complications, such as blindness.

Symptoms of Behcet’s Disease

Behcet’s Disease is common in the Middle East, and in Asia. It is rare in the United States. In Middle Eastern and Asian countries, the disease affects more men than women. In the United States, it affects more women than men.

The exact cause of Behcet’s Disease is unknown. Behcet’s Disease tends to develop in people in their 20′s or 30′s, but all age groups may be affected. Behcet’s Disease is an autoinflammatory disease resulting in damage to blood vessels throughout the body, particularly veins. It is a form of vasculitis (an inflammation of the blood vessels). Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat, and pain.

Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful “foreign” substances. In an autoimmune reaction, the immune system mistakenly attacks and harms the body’s own tissues.

Behcet’s Disease is not contagious; it does not spread from one person to another and affects each person differently. The four most common symptoms are mouth sores, genital sores, inflammation of the internal portions of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) may cause blurred vision, pain and redness. Other symptoms of the disease may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs.

Behcet’s Disease has the ability to involve blood vessels of nearly all sizes and types, in veins and arteries. Because of the diversity and size of the blood vessels affected, manifestations of Behcet’s may occur at many sites throughout the body.

However, the disease seems to target certain organs and tissues, as described below:

Eye

Behcet’s may cause anterior uveitis (inflammation in the front of the eye) or posterior uveitis (inflammation in the back of the eye). At times, both portions of the eye may be affected.
Anterior uveitis results in pain, blurry vision, light sensitivity, tearing or redness of the eye. Many cases occur in healthy individuals. It may affect one or both eyes. It is the most common form of uveitis, predominantly occurring in the young and middle aged population, but may be associated with rheumatic, gastrointestinal, skin, lung and infectious diseases.
Posterior uveitis is the least common form of uveitis. Symptoms may include blurred vision, floaters, eye pain, redness and sensitivity to light. Posterior uveitis often involves the retina and the choroid. There are many infectious and non-infectious causes to posterior uveitis. This type of uveitis can only be detected during an eye examination and must be treated in order to avoid serious complications and loss of vision.

Mouth

Painful sores in the mouth called “aphthous ulcers” (known as oral aphthosis [af-THO-sis] and aphthous stomatitis) affect almost all patients with Behcet’s disease. Individual sores or ulcers are usually identical to canker sores, which are common in many people. These sores are usually a result of minor trauma. They are often the first symptom that a person notices and may occur long before any other symptoms appear. However, the lesions are more numerous, more frequent, and often larger and more painful. Aphthous ulcers can be found on the lips, tongue, and inside of the cheek. Aphthous ulcers may occur individually or in clusters, but occur in virtually all patients with Behcet’s. The sores usually have a red border and several may appear at the same time. They may be painful and can make eating difficult. Mouth sores go away in 10 to 14 days but often come back. Small sores usually heal without scarring, but larger sores may scar.

Skin

Skin problems are a common symptom of Behcet’s disease. Skin sores often look red or resemble pus-filled bumps or a bruise. The sores are red and raised, and typically appear on the legs and on the upper torso. In some people, sores or lesions may appear when the skin is scratched or pricked. When doctors suspect that a person has Behcet’s disease, they may perform a pathergy test, in which they prick the skin with a small needle; 1 to 2 days after the test, people with Behcet’s disease may develop a red bump where the doctor pricked the skin. However, only half of the Behçet’s patients in Middle Eastern countries and Japan have this reaction. It is less commonly observed in patients from the United States, but if this reaction occurs, then Behçet’s disease is likely.
Pustular skin lesions that resemble acne, but can occur nearly anywhere on the body. This rash is sometimes called “folliculitis”.
Skin lesions called erythema nodosum: red, tender nodules that usually occur on the legs and ankles but also appear sometimes on the face, neck, or arms. Unlike erythema nodosum associated with other diseases (which heal without scars), the lesions of Behcet’s Disease frequently ulcerate.

Lungs

Aneurysms (outpouchings of blood vessel walls, caused by inflammation) of arteries in the lungs, which may rupture and lead to lung hemorrhage.

Joints

Arthritis or “arthralgias” -inflammation of the joints, occurs in more than half of all patients with Behcet’s Disease. Arthritis causes pain, swelling, and stiffness in the joints, especially in the knees, ankles, wrists, and elbows. Arthralgias from Behcet’s Disease last days to weeks and may not cause permanent damage to the joints.

Brain

Behcet’s Disease affects the central nervous system in about 23 percent of all patients with the disease in the United States. The central nervous system includes the brain and spinal cord. Its function is to process information and coordinate thinking, behavior, sensation, and movement. Behcet’s Disease can cause inflammation of the brain and of the thin membrane covering and protecting the brain and spinal cord. This condition is called meningoencephalitis.Patients with meningoencephalitis may develop fever, headache, stiff neck, and difficulty coordinating movement, and should report any of these symptoms to their doctor immediately. If the condition is left untreated, a stroke (blockage or rupture of blood vessels in the brain) can result.

Central nervous system involvement is one of the most dangerous manifestations of Behcet’s. The disease tends to involve the “white matter” of the brain and brainstem, and may lead to headaches, confusion, strokes, personality changes, and (rarely) dementia. Behcet’s may also involve the protective layers around the brain (the meninges), leading to meningitis. Because the meningitis of Behcet’s Disease is not associated with any known infection, it is often referred to as “aseptic” meningitis.

Genitals

Genital sores affect more than half of all patients with Behcet’s Disease. The sores may be painful. After several outbreaks, they may cause scarring.
Males — painful genital lesions may form on the scrotum, similar to oral lesions, but deeper.
Females — painful genital ulcers may develop on the vulva and vagina.

Gastrointestinal

Behcet’s Disease causes inflammation and ulceration (sores) throughout the digestive tract. The lesions are similar to the aphthous lesions in the mouth and genital area.
Ulcerations may occur anywhere in the gastrointestinal tract from the mouth to the anus. This may lead to abdominal pain, diarrhea, and/or bleeding. Because these symptoms are similar to symptoms of other diseases of the digestive tract, ulcerative colitis and Crohn’s Disease are part of the differential diagnosis. Careful evaluation is essential to rule out other diseases.

What Causes Behcet’s Disease?

The cause of Behcet’s Disease is not known. Some medical professionals believe that it is an autoimmune disorder. With an autoimmune disorder, the body attacks its own healthy cells.

A combination of genetic influences and environmental factors may also lead to Behcet’s Disease.

Risk Factor of Behcet’s Disease?

Factors that may increase your risk of Behcet’s include:

Age. Behcet’s disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition.
Location. Although the disease occurs worldwide, people from countries in the Middle East and Far East, including Turkey, Iran, Japan and China, are more likely to develop Behcet’s.
Sex. While Behcet’s disease occurs in both men and women, the disease is usually more severe in men.
Genes. Having certain genes is associated with a higher risk of developing Behcet’s.

How Behcet’s Disease Diagnosed?

Diagnosing Behcet’s disease can be a challenge; instead of performing a diagnostic test, your physician may take a multifaceted approach. He or she may take laboratory samples and assess your symptoms. Doctors often look for people who have had recurrent mouth sores that appeared at least three times in one year along with other symptoms such as genital sores or eye problems.

Complications of Behcet’s Disease

Behcet’s disease typically comes and goes in unpredictable cycles. Symptoms may become less severe over time.

Though treatment can’t cure Behcet’s disease, it often can control signs and symptoms and reduce the risk of complications. For instance, untreated uveitis can lead to decreased vision or even blindness. People with eye signs and symptoms of Behcet’s disease should be carefully monitored by an eye doctor because treatment can help prevent this complication. Other complications of Behcet’s disease depend on the specific set of signs and symptoms you’re experiencing.

How Behcet’s Disease Treated?

A variety of medications can treat Behcet’s Disease and alleviate pain and other symptoms.

A doctor may prescribe medications to take only during flare-ups. Sometimes people with more severe symptoms are placed on a long-term medication or medications as well. Age and sex are two factors a physician will probably consider when devising a treatment plan for patients with Behcet’s Disease.

Some topical medications can be used to treat Behcet’s Disease.

A doctor may also prescribe a mouth rinse containing corticosteroids. This can lessen the pain from mouth sores. Eye drops containing corticosteroids or other medications can help soothe redness or pain in the eyes if they are affected.