What is Behçet’s disease?
Behçet’s disease, also called Behçet syndrome or Silk Road disease, is a type of vasculitis—a rare, long-term illness that causes inflammation in blood vessels throughout the body. It’s believed to involve an abnormal immune system response and can lead to a range of symptoms that often come and go in flares, with periods of relief in between.
Because this condition is rare, affects many parts of the body, and is linked to immune system dysfunction, it’s important to seek guidance from experts in autoimmune disease care. Penn Medicine offers specialized, team-based treatment for Behçet’s disease, making it a strong choice for diagnosis, management, and long-term support.
Symptoms of Behçet’s disease
Common symptoms of Behçet’s disease include:
- Painful mouth sores that look like canker sores and come back often
- Genital sores that cause significant discomfort and may leave scars
- Skin problems such as bumps that look like acne or painful red lumps, usually on the legs
- Eye inflammation with redness, pain, and blurry vision
- Joint pain and swelling, often in the knees, ankles, elbows, or wrists
- Blood vessel problems including swelling, redness, and blood clots
- Digestive issues that may include stomach pain, diarrhea, nausea, vomiting, or blood in the stool
- Nervous system symptoms such as headaches, trouble thinking clearly, poor balance, or, in severe cases, stroke
What causes Behçet’s disease?
The exact cause of Behçet’s disease is not fully understood, but experts believe it develops from a mix of genetics, environmental triggers, and problems with the immune system. People who have the HLA-B51 gene have an increased risk for Behçet’s disease. Infections or other environmental factors may trigger the disease. Once the condition emerges, the immune system becomes overactive and causes inflammation in blood vessels throughout the body.
Behçet’s disease is sometimes called Silk Road disease because it’s more common in people from countries along the ancient Silk Road trade routes, including parts of the Middle East, Central Asia, and East Asia. Scientists believe that both genes and human migration patterns may explain this connection.
Diagnosing Behçet’s disease
There is no single test to confirm Behçet’s disease. Doctors make the diagnosis by reviewing symptoms, doing a physical exam, and ordering blood tests to rule out other conditions. They look for a pattern of repeated mouth sores along with at least two other signs, such as genital ulcers, eye inflammation, or skin changes. They may also perform a pathergy test, which checks for an unusual skin reaction to a minor poke or needle stick.
Treating Behçet’s disease
There’s no cure for Behçet’s disease, but treatment can help manage symptoms, reduce inflammation, and prevent serious complications. Treatment plans are tailored to each person based on symptom severity and which body parts are affected. Doctors may use anti-inflammatory drugs like steroids to control flares, as well as immunosuppressants to calm the immune system. Some people may also benefit from biologic therapies that target specific parts of the immune response. Supportive treatments, like special eye drops, mouth rinses, or skin creams, can ease discomfort during flares. In some cases, where there are serious or life-threatening complications, surgery may be needed.
Experienced care for rare autoimmune conditions
Behçet’s disease is complex, and symptoms can vary from person to person, so it’s important to seek care from experts who understand this rare condition. Penn Medicine is a trusted choice because our specialists are experienced in diagnosing and treating all types of vasculitis, including Behçet’s. Our teams bring together different types of doctors, such as rheumatologists and eye specialists, who work together to manage all aspects of your condition.
As a leading academic medical center, we provide Behçet’s disease care that’s grounded in the latest research. You’ll have access to advanced tests and may even qualify for clinical trials that offer new treatments.