Behcet's disease, also known as Behcet's syndrome, is a long-term auto-inflammatory disorder that affects the body's blood vessels.
These tubes, such as the arteries and veins, carry the blood around the body.
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Here are some key points about Behcet's disease. More detail and supporting information is in the main article.
- Behcet's disease is a rare condition that is most commonly seen in Turkey. However, it is now accurately diagnosed and reported in other countries throughout the world.
- According to the American Behcet's Disease Association, the prevalence of Behcet's disease in Turkey is as high as 400 cases per 100,000 persons
- In comparison, prevalence of Behcet's disease in the United States is 1 case per 170,000 persons
- The condition is not contagious and cannot be spread from person to person
- Behcet's disease affects everyone differently, and symptoms may intensify and weaken over a period of weeks or longer.
Causes of Behcet's disease
While the exact cause of Behcet's disease is unknown, certain groups of people are at a higher risk of developing the disease than others.
Although all ages and sexes are at risk of developing Behcet's disease, people are most commonly affected in their 20s and 30s. Men typically experience more severe symptoms than women.
Ethnicity and geographic location may play a role in how likely a person is to develop Behcet's disease. The condition is most common in men from the Middle East and Asia, and women from the United States, other Western Countries, Japan, and Korea.
There may be a genetic or inherited component to the disease. Behcet's disease could also be linked to bacteria, viruses, or environmental factors. More research is needed, however, before these suggestions can be confirmed.
Symptoms of Behcet's disease
Behcet's disease affects everyone differently. People with the disease may experience symptom flares, in which the symptoms wax and wane over a period of weeks or longer. The symptoms of Behcet's disease may also go away without treatment.
The condition causes a wide range of symptoms affecting different areas of the body. These symptoms include the following.
Sores in the mouth are one of the most common symptoms of Behcet's disease.
Typically, painful sores in the mouth are the first symptom of Behcet's disease. The disease can cause a single ulcer to develop or more than one sore.
These sores can from anywhere in the mouth, Specific areas include the tongue, lips, gums, tonsils, lining of the cheeks, roof of the mouth, and the back of the throat.
Mouth sores caused by Behcet's disease can be:
- Shallow or deep
- Round or oval
- White or yellow base with a red halo surrounding the sore
- Ranging in size from 1 to 20 millimeters
Sores in the mouth often go away within 10 to 20 days with occasional scarring.
According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, over half of people with Behcet's disease will develop genital lesions. This figure includes both males and females.
Men may have ulcers on the scrotum and penile shaft or head. At times, these lesions are associated with another condition called epididymitis, inflammation of the tubes that carry sperm. Women may experience lesions on the vulva, vagina, and cervix
These sores typically present as:
- Painful red, open sores
- May be large and deep
When these sores heal, scarring often affects the area.
People with Behcet's disease may experience skin problems called erythema nodosum. This inflammatory skin response causes the skin to develop red and tender nodules that are often ulcerated.
These lesions may bear a resemblance to pus-filled bumps or bruises. Other lesions associated with Behcet's disease include acneiform nodules, pseudofolliculitis, and papulopustular lesions.
Those affected with Behcet's disease often experience inflammation of the middle layer of the eyes. This condition is known as uveitis.
Anterior uveitis affects the front of the eye. Common symptoms include pain, blurry vision, sensitivity to light, and excessive tear production. A layer of pus may be seen within the eye.
Posterior uveitis affects the back of the eye and retina. Common symptoms include blurry vision, floaters, pain, redness, and sensitivity to light.
Eye problems caused by Behcet's disease can cause serious complications if they go untreated, including blindness or partial loss of vision.
Inflammation of the veins and arteries can lead to serious complications such as blood clots, aneurysm, and narrowed or blocked vessels.
People with Behcet's disease may experience a condition called thrombophlebitis, with symptoms such as redness, pain, warmth, and swelling of an extremity due to the presence of a blood clot. Chest murmurs have also been reported.
Behcet's disease can cause inflammation in the joints, leading to pain, swelling, and stiffness.
One or more joints may be affected by joint inflammation related to Behcet's disease. The pain, swelling, and stiffness typically goes away over the course of a few weeks.
The joints most commonly affected include the knees, ankles, wrists, and elbows. Some people affected by Behcet's disease will experience low back or buttock pain caused by inflammation to a joint in the pelvis.
No permanent joint damage is experienced with Behcet's-related arthritis.
At times, ulcers may form anywhere in the digestive system from the mouth to the anus. Patients affected in this area can experience symptoms such as lack of appetite, pain, vomiting, diarrhea, and rectal bleeding.
A rare condition known as Budd-Chiari syndrome may develop in some patients. In this condition, the vein that carries blood away from the liver is blocked.
Although rare, the lungs may be affected by Behcet's disease. The condition can cause symptoms such as coughing, shortness of breath, and aneurysms in the pulmonary artery.
Central nervous system problems
Behcet's disease can lead to inflammation of the brain and brain stem. This inflammation can cause symptoms such as headaches, confusion, strokes, personality changes, disorientation, fever, poor balance, and memory loss.
Symptoms that patients should report to the healthcare team at once include:
- Stiff neck
- Difficulty with coordination
These symptoms can indicate inflammation of the brain stem. Untreated symptoms can result in a stroke.
In some cases, patients may exhibit cardiac or renal disease.
Additionally, some patients may develop mouth and genital ulcers with inflamed cartilage, referred to as MAGIC syndrome. This symptom is most likely to occur in patients with both Behcet's disease and another rare disorder called relapsing polychondritis. This condition causes cartilage and other connective tissue in the body to become inflamed.
Diagnosis of Behcet's disease
Because there is not a single test to diagnose Behcet's disease, doctors need to rule out any conditions that mimic the disease.
Doctors need to make sure that symptoms are not caused by another condition before being able to diagnose Behcet's disease.
The American Behcet's Disease Association explain that the International Clinical Criteria for Behcet's disease diagnosis require that certain symptoms must be present for a diagnosis to be made. A diagnosis requires:
- The presence of recurring mouth ulcers at least three times in one single year
In addition to the above, at least two of the criteria below must also be met:
- Recurring genital ulcers
- Eye sores confirmed by an eye exam
- Skin sores in adults who are not taking corticosteroids
- A positive pathergy test reading within 24-48 hours of the test
A pathergy test involves a doctor inserting a small, clean needle into the skin of the forearm. A positive result is given if a small, red bump forms 1 to 2 days after the needle has been inserted.
Treatments for Behcet's disease
Once a person has been diagnosed with Behcet's disease, their healthcare team will make recommendations about treatment options.
Topical therapy is medication that is applied to the surfaces of the body. Topical therapy for Behcet's disease may include the use of pain-relieving therapy, including corticosteroid rinses, gels, eye drops, and ointments. These medications may include other ingredients to provide pain relief.
Examples of medications include triamcinolone acetonide, betamethasone, and dexamethasone.
At times, it may be necessary to undergo treatment with drugs that work throughout the body. These drugs include:
- Colchicine, a medication used to prevent gout
- Medications to suppress the immune system such as azathioprine, cyclosporine, and cyclophosphamide
- Medications that change how the body's immune cells work
Additional medications may be recommended based on the symptoms that develop. Patients are advised to speak with their healthcare provider to discuss treatment options.
Healthcare providers may also recommend simple lifestyle modifications such as exercise and rest.