What Is Behcet's Disease?

Behcet's disease is a rare autoimmune disease that causes blood vessel inflammation—called vasculitis—throughout the body. All-over inflammation can lead to damage to blood vessels, and cause mouth sores, rashes, and eye problems. The severity of the disease varies person-to-person. Symptoms may come and go and the disease may go into periods of remission (where diseases stop or reduce activity) and periods of flare-ups (high disease activity). Fortunately, Behcet's can be managed with various treatments.

Here is what you need to know about Behcet’s disease, including symptoms, causes, diagnosis, and treatment.

Behcet's Disease Symptoms

The earliest symptoms of Behcet's are sores located inside of the mouth. They may appear like canker sores and heal within a few weeks. According to the American Behcet’s Disease Association, genital sores are the next common symptom. They affect up to 75% of the people with the condition. Sores can also appear in other places on the body, most commonly on the face and neck.

Behcet’s disease may also affect the eyes and cause:

• Vision problems
• Sensitivity to light
• Swelling in one or both eyes
• Eye redness

Additional symptoms of Behcet’s include:

• Painful and swollen joints
• Digestive problems, including diarrhea and abdominal pain
• Inflammation in the brain that leads to headaches
• Fatigue
• Skin lesions, often resembling acne

Lung involvement is a less common, but serious, symptom of Behcet’s disease. It presents with cough, dyspnea (shortness of breath), and sometimes pulmonary artery aneurysms. An aneurysm is a localized enlargement of an artery caused by the weakening of the artery’s wall. Aneurysms of the lungs can rupture and cause lung hemorrhaging (bleeding).

Causes

Exact causes of most types of vasculitis are not fully understood. Some types are related to genetic makeup and immune system diseases that attack the blood vessels by mistake. Immune system diseases that trigger this reaction are infections, blood cancers, and inflammatory conditions such as rheumatoid arthritis and lupus. Drug reactions are also triggers. Risk factors associated with vasculitis are smoking, hepatitis B and C infections, and other autoimmune diseases.

According to the Johns Hopkins Vasculitis Center, Behcet’s disease is one of the few types of vasculitis that has a known genetic disposition. The presence of a specific gene—HLA-B51—is associated with the development of Behcet’s. It should be noted, however, that simply having the gene does not mean a person will develop Behcet’s. HLA-B51 is only associated with 5% of Behcet's cases. As a result, researchers and healthcare providers think other factors—including infections and environmental exposures­—also play a part.

Diagnosis

There is no single lab test that can confirm a person has Behcet’s. Your healthcare provider will rely on signs and symptoms you are experiencing to make a diagnosis.

Because most people with the condition develop mouth sores, a diagnosis can be made based on the recurrence of mouth sores. Other symptoms that can help in making a Behcet’s diagnosis are:

• Skin sores
• Genital sores
• Eye inflammation

Additional testing your healthcare provider may request includes:

• Blood tests and other lab work to rule out other conditions
• Pathergy test: With this test, your healthcare provider will insert a sterile needle into the skin and examine the area a day or two later. If the test is positive for Behcet’s, a small red bump will form under the skin where the needle was injected. This is an indication the immune system is overreacting to a small injury.

Treatment

There is no cure for Behcet’s disease, but the condition is treatable, and treatment can help relieve symptoms. This will include a combination of drug therapies and lifestyle changes.

Drug Therapies

Research shows the management of Behcet’s disease has evolved considerably in recent years due to a better understanding of older drugs and the development of new therapies, such as biologic drugs. Medications used to treat Behcet’s include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, immunosuppressants, biological therapies, and topical therapies.

NSAIDs: NSAID therapy for Behcet’s disease can treat inflammation and joint pain.

Corticosteroids: These drugs reduce inflammation and are often used as systemic treatment (affecting the whole body), or as topical therapies, i.e. for treating mouth sores.

Immunosuppressants: The medications are also systemic treatments and help to reduce the activity of the immune system. Because Behcet’s is caused by an overactive immune system, these drugs can help reduce symptoms.

Biological therapies: Biologic drugs are newer, systemic therapies that target specific biological proteins that promote disease activity and cause symptoms. For example, biologics affect antibodies that lead to inflammation.

Topical therapies: These are medications applied to surfaces of the body. Topical treatments include topical pain relief creams, corticosteroid rinses, and eye drops. 

Your healthcare provider may recommend additional medications based on your symptoms. You should discuss treatment options and any concerns you have about medications with your healthcare provider.

Lifestyle

Some lifestyle strategies may help reduce symptoms, including diet, exercise, and stress management

Diet: There is no evidence a specific diet can improve symptoms of Behcet’s disease, but a healthy and balanced diet will improve overall health and potentially reduce inflammation. Some foods may trigger inflammation and Behcet’s symptoms. One study reported in 2016 shows certain food items were related to mouth sore symptoms in Behcet's, including pineapple, some cheeses, some types of nuts, and lemon.

Exercise: Exercise may help to reduce pain and complications of Behcet’s disease, according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). Moderate activity, such as walking, is safe for most people, but you should talk to your healthcare provider before starting any exercise program to make sure you are working out safely with Behcet’s disease.

Managing stress: Stress is a trigger for most autoimmune diseases, so learning to relax may help to reduce symptoms and the number of flare-ups you have.

Potential Complications

Many of the symptoms of Behcet’s disease are manageable and rarely pose permanent and life-threatening complications. But treatment is important to avoid complications. For example, untreated inflammation may put a person at risk for vision problems. And because Behcet’s affects the blood vessels, serious vascular problems can occur. For example, inflammation of the arteries and veins can lead to blood clots. Stroke is another potential complication that occurs when blood flow to the brain stops.

A Word From Verywell

Living with Behcet’s disease has challenges, and fatigue, pain, emotional stress, and medication side effects can affect your well-being and many parts of your life. It might be helpful to talk to loved ones about your worries or to connect with others in a support group or even to talk to a mental health professional.

The outlook for most people with Behcet’s disease can be good and having the condition usually does not affect life expectancy. But you have to follow your healthcare provider’s treatment advice, take medications as prescribed, and maintain an active and healthy lifestyle to keep feeling your best and living a good, full life.