Behcet’s Syndrome

​Behcet’s Syndrome, also called Behcet’s Disease, is a rare disease that mainly affects young adults. It causes inflammation in many parts of the body including the eyes, mouth, skin, genitals, joints, blood vessels and nervous system.

Behcet’s Syndrome can affect both males and females of all ages. The first symptoms often appear between the age of 20 and 30. The disease tends to be more severe in men and when the onset is at an earlier age.

Signs and symptoms of Behcet’s Syndrome may vary from person to person and also depending on which parts of the body are affected. Organs that are commonly affected by Behcet’s Syndrome include the following:

• Mouth
  Presence of painful mouth ulcers is the most common sign of Behcet’s Syndrome. The mouth ulcers can occur on the inner surface of the lips, gums, cheeks or tongue. They usually heal within 7 to 10 days but often recur.

• Skin
  Skin rashes are also common in Behcet’s Syndrome. Some patients develop acne-like rashes on their body, while others may develop red, raised and tender nodules on their shins. Skin rashes may clear up on their own within 10 to 14 days, but they usually recur.

• Genitals
  Patients with Behcet’s Syndrome may develop painful ulcers on their genitals. These ulcers most commonly occur on the penis or the vulva, as round, red and ulcerated lesions. They are not a form of sexually transmitted disease.

• Eyes
  Behcet’s Syndrome may cause inflammation of the eyes, known as uveitis, which presents with redness, pain and blurred vision. If not treated promptly, it can lead to blindness.

• Joints
  Behcet’s Syndrome can cause arthritis. The affected joints become swollen, red and painful due to inflammation. Joints that are commonly affected are the knees, ankles, elbows and wrists.

• Blood vessels
  Inflammation of the veins and arteries may occur, resulting in redness, pain and swelling in the arms or legs. Occasionally blood clots can occur in the vessels (thrombosis).

• Digestive system
  A variety of signs and symptoms may affect the digestive system, including abdominal pain, diarrhea or bleeding from the intestines.

• Brain
  Inflammation in the brain and nervous system may occur, causing headaches, fever, disorientation, poor balance and stroke.

​Currently there is no preventive measure for the disease.

​The exact cause is unknown. Many believe it is a form of autoimmune disorder in which the body’s immune system becomes dysfunctional and attacks its own healthy cells. Both genetic and environmental factors such as repeated infections have been linked to the possible causes of Behcet’s Syndrome.

​The symptoms of Behcet’s Syndrome can mimic many other diseases and therefore these conditions need to be excluded before a diagnosis of Behcet’s Syndrome can be made. There is no specific blood test that can diagnose Behcet’s Syndrome. Hence the diagnosis is based on a set of symptoms and signs, including recurrent mouth ulcers at least three times a year, in addition to two of the following:

• Eye problems – as confirmed by an eye specialist
• Skin rashes
• Genital ulcers
• Positive pathergy test, which is a test to indicate whether the immune system is over-reacting to a minor injury.

​There is currently no cure for Behcet’s disease. Treatment strategies focus on controlling inflammation, alleviating the specific symptoms experienced by the patient and preventing serious complications. In mild cases, treatment is only necessary during acute flares and no treatment is necessary in between flares. In severe cases, medication may be necessary over a period of time. Treatment usually includes one or more of the following:

• Topical creams, gels and ointments
  These are usually applied on the skin rashes or mouth / genital ulcers. They usually contain steroid that reduces the inflammation and an anesthetic drug that relieves pain.

• Mouth rinses
  These reduce the inflammation in the mouth and ease the pain and discomfort.

• Eye drops
  Eye drops containing steroids or other anti-inflammatory medications can relieve pain and redness in the eyes.

• NSAIDs and COX-2 inhibitors
  These oral drugs reduce pain and swelling of the joints, and are particularly useful in arthritis. However, long-term use can cause kidney damage, gastric discomfort and even bleeding especially in patients with a previous history of stomach ulcers. These drugs should also be used with care in patients who have heart disease, hypertension or a history of stroke.

• Colchicine, oral steroids and other immunosuppressive drugs
  Colchicine is usually prescribed for arthritis and the prevention of recurrent oral or genital ulcers or other skin manifestations. Medication such as prednisolone is often used successfully in patients with moderate or severe Behcet’s Syndrome. It reduces pain and inflammation. Often steroids are used in combination with other immunosuppressive drugs such as cyclophosphamide, methotrexate, azathioprine, mycophenolate or cyclosporine. Since these medications suppress the immune system, there is a risk of infection. Other drugs that have been used to treat Behcet’s Syndrome include interferon-alpha, biologic agents (e.g. adalimumab, etanercept and infliximab) and apremilast.

• Rest and exercise
  Sufficient rest is important when a patient is suffering from a flare. During the period of remission, doctors may recommend a regimen of moderate exercise such as swimming or walking, to help keep the joints strong and flexible.

​Please consult your rheumatologist before any elective surgery. Medication adjustment may be required in preparation for the surgery.

​Please consult your surgeon or rheumatologist for your post-surgical care. Any medication that is temporarily suspended before the surgery may have to be restarted.