Behcet's (beh-CHETS) disease, also called Behcet's syndrome, is a rare disorder that causes blood vessel inflammation throughout your body.
The disease can lead to numerous signs and symptoms that can seem unrelated at first. They can include mouth sores, eye inflammation, skin rashes and lesions, and genital sores.
Treatment involves medications to reduce the signs and symptoms of Behcet's disease and to prevent serious complications, such as blindness.
Behcet's disease symptoms vary from person to person, can come and go or become less severe over time. Signs and symptoms depend on which parts of your body are affected.
Areas commonly affected by Behcet's disease include:
Make an appointment with your doctor if you notice unusual signs and symptoms that might indicate Behcet's disease. If you've been diagnosed with the condition, see your doctor if you notice new signs and symptoms.
Behcet's disease might be an autoimmune disorder, which means the body's immune system mistakenly attacks some of its own healthy cells. It's likely that genetic and environmental factors play a role.
The signs and symptoms of Behcet's disease are considered to be due to inflammation of the blood vessels (vasculitis). The condition can involve arteries and veins of all sizes, damaging them throughout the body.
Several genes have been found to be associated with the disease. Some researchers believe a virus or bacterium can trigger Behcet's disease in people who have certain genes that make them susceptible to Behcet's.
Factors that might increase your risk of Behcet's include:
Complications of Behcet's disease depend on your signs and symptoms. For instance, untreated uveitis can lead to decreased vision or blindness. People with eye signs and symptoms of Behcet's disease need to visit an eye specialist (ophthalmologist) regularly because treatment can help prevent this complication.
No tests can determine whether you have Behcet's disease, so your doctor will rely primarily on your signs and symptoms. Because nearly everyone with the condition develops mouth sores, mouth sores that have recurred at least three times in 12 months are generally necessary for a diagnosis of Behcet's disease.
In addition, a diagnosis of Behcet's disease requires at least two additional signs, such as:
Tests you might need include:
There's no cure for Behcet's disease. If you have a mild form, your doctor might suggest medications to control the pain and inflammation of flares. You might not need medication between flares.
For more-severe signs and symptoms, your doctor might prescribe medications to control the Behcet's disease throughout your body, in addition to medications for flares.
Medications to control signs and symptoms you have during flares might include the following:
If topical medications don't help, your doctor might recommend a drug called colchicine (Colcrys, Mitigare) for recurring oral and genital sores. Joint swelling also might improve with colchicine.
Severe cases of Behcet's disease require treatments to control damage from the disease between flares. If you have moderate to severe Behcet's disease, your doctor might prescribe:
Corticosteroids to control inflammation. Corticosteroids, such as prednisone, are used reduce the inflammation caused by Behcet's disease. Doctors often prescribe them with another medication to suppress the activity of your immune system.
Side effects of corticosteroids include weight gain, persistent heartburn, high blood pressure and bone thinning (osteoporosis).
Medications that alter your immune system's response. Interferon alfa-2b (Intron A) regulates the activity of your immune system to control inflammation. It may be used alone or with other drugs to help control skin sores, joint pain and eye inflammation in people with Behcet's disease. Side effects include flu-like signs and symptoms, such as muscle pain and fatigue.
Medications that block a substance called tumor necrosis factor (TNF) are effective in treating some of the signs and symptoms of Behcet's, especially for people who have more-severe or resistant symptoms. Examples include infliximab (Remicade) and adalimumab (Humira). Side effects might include headache, skin rash and an increased risk of infections.
The unpredictability of Behcet's disease can make it particularly frustrating. Taking good care of yourself might help you cope. In general, try to:
You're likely to start by seeing your primary care doctor. He or she may refer you to a doctor who treats arthritis and other rheumatic illnesses (rheumatologist).
Depending on your signs and symptoms, you might also need to see an ophthalmologist for eye problems, a gynecologist or urologist for genital sores, a dermatologist for skin problems, a gastroenterologist for digestive difficulties, 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 come along to help you remember the information you receive.
For Behcet's, questions to ask your doctor include:
Your doctor is likely to ask you questions, such as: