Behçet’s Disease

What is Behçet’s disease?

Behçet’s disease, also known as Behçet’s syndrome, was recorded by a Turkish dermatologist named Dr. Hulusi Behçet. It is a chronic condition that commonly occurs in Japan, Asia, and the Middle East (where it affects more men than women), but it is actually quite rare in the US (where it affects more women than men), and it usually starts in someone’s twenties or thirties. Behçet’s disease causes chronic inflammation which manifests as ulcers (commonly referred to as canker sores). These ulcers most often appear in the mouth and on the genitals. Inflammation also commonly affects the eye and skin. Sometimes, this inflammation attacks the joints, and, in rare cases, the digestive system, brain, and/or spinal cord.

What causes Behçet’s disease?

Simply put, we don’t know. Behçet’s disease is a form of vasculitis (inflammation of the blood vessels) where the body attacks its own blood vessels. This makes it an auto-inflammatory condition. But, no one knows what causes this autoimmune reaction to start in the first place. From what researchers can determine, there are two factors that seem linked to the disease. One is immune system irregularities, which could be inherited, and make it more likely that a person would get Behçet’s disease. The other factor is an environmental trigger that starts the auto-inflammatory process in a person who is already predisposed to get Behçet’s disease.

Is there a test that leads to diagnosis of Behçet’s disease (BD)?

No. Since Behçet’s symptoms tend to overlap with many other diseases, tests are done to rule out other explanations. Misdiagnosis is not uncommon. Less than half of people with BD have a positive reaction to a pathergy (skin prick) test, and there is believed to be a correlation between BD and the gene HLA-B51. However, you can have negative results for both and still have Behçet’s disease.

Therefore, diagnosis of Behçet’s disease comes after ruling out all other possibilities and:

• Having three instances of mouth sores within 12 months
• Two other symptoms: genital sores that come back, certain skin lesions (see below), positive result from a pathergy test, or eye inflammation

What are common symptoms of Behçet’s disease?

Because Behçet’s disease can affect large and small blood vessels anywhere in the body, its course can differ greatly from person to person. Symptoms typically come and go, in periods of flares and remission, and can stay for a few weeks to many months. The tricky part with Behçet’s is that each flare can be caused by different symptoms.

Let’s start with the most common Behçet’s disease symptoms:

• Painful mouth sores (also called aphthous stomatitis) are usually the first symptom and most common sign of BD.
• Genital sores look similar to mouth sores and are present in the majority of patients with BD, affecting men and women equally.
• Skin lesions are typically acne-like or red, tender bumps on the legs and torso.
• Uveitis (eye inflammation) characterized by blurred vision, pain, and redness. If left untreated, it can lead to blindness.
• Arthritis that typically affects the knees, ankles, elbows, and wrists, but usually doesn’t cause lasting damage.

In more serious and rare cases, Behçet’s disease can cause:

• Ulcers in the digestive system
• Blood clots
• Inflammation of the brain

What are treatment options for Behçet’s disease (BD)?

Since Behçet’s disease can affect the body in many different ways, usually many types of doctors are involved in care. Since there is no cure, the goal of treatment is symptom control and creating longer periods of remission. A rheumatologist is often the primary doctor in charge of coordinating care.

Common types of treatment are:

• Topical medicine to heal sores faster and help relieve pain.
• Corticosteroids to help control inflammation and reduce pain.
• Immunosuppressive drugs to help control systemic manifestations of BD, especially chronic eye or brain inflammation.
• Biologics, which target specific parts of the immune system, have shown promise in difficult-to-control BD.
• Of course, leading a healthy lifestyle with the right balance of rest and exercise, along with a healthy diet, are essential.

Where can I find more information about Behçet’s disease?

• John Hopkin’s Medicine Vasculitis Center
• National Institute of Arthritis and Musculoskeletal and Skin Diseases
• American Behcet’s Disease Association
• NORD