Behcet's disease or Behcet's syndrome is a rare autoimmune disease that causes inflammation of blood vessels throughout the body. The disease was first described in the 1930s by a Turkish dermatologist, Hulusi Behcet, who noted a set of three symptoms: canker sores, genital ulcers and recurrent eye inflammation. The disease can produce symptoms in many parts of the body, including the mouth.
Who Develops Behcet's Syndrome?
The cause of Behcet's disease is not known. Johns Hopkins Medicine reports that the HLA-B51 gene is found in some patients with Behcet's syndrome, though the presence of the gene alone does not cause Behcet's. Only 5 percent of cases have shown to be familial, and the disease is not contagious. Therefore, scientists are researching potential infectious or environmental causes for the syndrome, according to a study published in Current Opinion in Rheumatology.
Found in populations across the world, this disease most often affects individuals in the Mediterranean, the Middle East and East Asia. According to the American Behcet's Disease Association, there are approximately 16,000 to 20,000 cases in the United States, or approximately one case out of every 170,000 people, categorizing it as a rare disease. In Turkey, where the disease is more common, men are more frequently affected than women. In the United States, women may be slightly more susceptible. The disease can develop in individuals of any age, though it most frequently occurs in young adults in their 20s and 30s.
Diagnosis and Symptoms
When diagnosing Behcet's disease, physicians and dentists will look for a number of key symptoms in their patients, as described by the National Institutes of Health:
- Mouth sores. The majority of Behcet's patients will experience round sores in the lining of the mouth. These are often recurrent and may be the first symptom to appear, which is why it's critical to communicate with your dentist about any problems you have with frequent mouth sores.
- Genital sores. Patients may also experience genital sores that appear similar to the mouth sores. They may be shallow or deep lesions with reddish borders.
- Eye inflammation. Some patients experience eye pain, blurred vision, sensitivity to light, tears and eye redness. In severe cases, the inflammation may result in blindness.
- Skin lesions. Common effects of Behcet's disease on the skin include acne-like bumps, nodules or ulcers with or without pain. A doctor might conduct a positive pathergy test by seeing if a red bump or sore develops when the skin is scratched or pricked with a needle, explains the Cleveland Clinic.
- Joint pain. Inflammation or swelling in the joints may cause pain in the ankles, knees, elbows and hips, but the joints are not often permanently damaged.
- Inflamed veins. Veins throughout the body — both deep veins and those close to the skin — may form clots. This can lead to severe issues with the functioning of the heart, brain or lungs.
Diagnosing Behcet's syndrome includes noting the above findings, especially how frequently a patient experiences mouth sores. Because there is no single lab test that can identify Behcet's disease, doctors will also work to rule out other causes for the sores, including lupus and Crohn's disease, according to the Cleveland Clinic.
Treatment depends on the severity of the illness. While the disease cannot be cured, your doctor may prescribe medications to help control the symptoms. The Mayo Clinic states that corticosteroids are often used to decrease inflammation and pain. Colchicine can help with mouth and genital sores, as can a medicated mouthrinse. Other medications suppress the immune system, such as infliximab.
Because it is a chronic disease, symptoms of Behcet's syndrome may appear and disappear even while a patient undergoes treatment. The Cleveland Clinic notes that only 4 percent of cases are fatal, usually due to intestinal holes, strokes or aneurysms. The key to effectively managing the disease is consulting a team of dentists and physicians to determine the best treatment options to minimize the symptoms.