Behcet (beh-CHET) disease, also called Behcet syndrome, is a rare condition. It causes blood vessel swelling, called inflammation, throughout the body.
Behcet disease symptoms can seem like they aren't related at first. They can include mouth sores, eye irritation and swelling, skin rashes and sores, and genital sores.
Treatment involves medicines to ease symptoms of Behcet disease and to prevent serious complications, such as blindness.
Behcet disease symptoms vary from person to person. Symptoms can come and go or become less serious over time. Symptoms depend on which parts of the body the condition affects.
Behcet disease most often affects the following:
Mouth. Painful mouth sores that look like canker sores are the most common sign of Behcet disease. They begin as raised, round sores in the mouth. They quickly turn into painful ulcers.
The sores most often heal in 1 to 3 weeks. But they often come back.
Make an appointment with your healthcare professional if you have symptoms that might be Behcet disease. If you've been diagnosed with the condition, see your health professional if you get new symptoms.
Behcet disease might be an autoimmune disorder, which means the body's immune system attacks some of its own healthy cells by mistake. It's likely that gene changes and factors in the environment play a role.
Experts believe that swelling of the blood vessels, called vasculitis, causes the symptoms of Behcet disease. The condition can involve arteries and veins of all sizes. Vasculitis can damage blood vessels all through the body.
Some genes might make people more likely to get Behcet disease. Some researchers think that a germ can trigger the disease in people who have these genes.
Factors that might increase your risk of Behcet disease include:
Complications of Behcet disease depend on symptoms. For instance, untreated uveitis can lead to loss of vision or blindness. If you have eye symptoms of Behcet disease, see an eye specialist, called an ophthalmologist, regularly. Treatment can help prevent this complication.
No tests can show that you have Behcet disease. So your healthcare professional mainly makes the diagnosis based on your symptoms. Nearly everyone with the condition gets mouth sores. So mouth sores that come back at least three times in 12 months often are needed for a diagnosis of Behcet disease.
Also, a diagnosis of Behcet disease needs at least two other symptoms, such as:
Tests to help diagnose Behcet disease include:
There's no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time.
For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares.
Medicines to manage symptoms during flares might include:
If topical medicines don't help, you might take a medicine by mouth called colchicine (Colcrys, Mitigare, others). This is for mouth and genital sores that come back. Colchicine also might ease joint swelling.
More recently, the U.S. Food and Drug Administration approved apremilast (Otezla) for the treatment of mouth sores caused by Behcet disease. Side effects may include weight loss and depression.
If you have more-serious Behcet disease, you may need treatments to lessen damage from the disease between flares. Your healthcare professional might prescribe:
Corticosteroids to manage swelling and irritation, called inflammation. Corticosteroids, such as prednisone (Rayos), ease the inflammation caused by Behcet disease. Healthcare professionals often prescribe corticosteroids with another medicine to make the immune system less active.
Side effects of corticosteroids include weight gain, heartburn, high blood pressure and bone thinning, called osteoporosis.
Medicines that make the immune system less active. Medicines that keep the immune system from attacking healthy tissues include azathioprine (Azasan, Imuran), cyclosporine (Gengraf, Neoral, others) and cyclophosphamide (Cytoxan).
These medicines can increase the risk of infection. They also can affect the liver and kidneys and cause low blood counts and high blood pressure.
Medicines that change how the immune system responds. Interferon alfa-2b (Intron A) may be used alone or with other medicines to help manage skin sores, joint pain and eye irritation in people with Behcet disease. Side effects include flu-like symptoms, such as muscle pain and fatigue.
Medicines that block a substance called tumor necrosis factor can treat some of the symptoms of Behcet disease. People who have worse symptoms or symptoms that resist treatment might take one of these medicines.
Medicines include infliximab (Remicade) and adalimumab (Humira). Side effects might include headache, skin rash and a higher risk of infections.
No one can predict the course of Behcet disease. And not knowing might be hard. Taking good care of yourself might help you cope with Behcet disease. Try to:
You're likely to start by seeing your primary healthcare professional. Your health professional might send you to a specialist who treats arthritis and other rheumatic illnesses, called a rheumatologist.
Depending on your symptoms, you also might need to see an ophthalmologist for eye problems, a gynecologist or urologist for genital sores, a dermatologist for skin problems, a gastroenterologist for digestive troubles, or a neurologist for symptoms that involve the brain or central nervous system.
Here's some information to help you get ready for your appointment.
Make a list of:
Ask a family member or friend to go with you to help you remember the information you receive.
For Behcet disease, questions to ask your healthcare team include:
Your healthcare team is likely to ask you questions, such as: