Behçet Disease

5 mins read

Introduction
Behçet disease also known as Behçet’s syndrome is a rare disorder that causes inflammation in blood vessels of whole body. Though the exact cause of disease is unknown, it is likely to be an autoimmune disorder. This implies that body’s immune system mistakenly attacks some of its own healthy cells. Both genetic and environmental factors could be accountable for Behçet.

Symptoms
Behçet symptoms vary from person to person. Behçet tends to disappear and recur by itself. The signs and symptoms one experiences depend on which parts of your body are affected by the inflammation of Behçet. Areas commonly affected by Behçet include:

  • Mouth: Painful mouth sores resembling canker sores is the most common symptom of Behçet. Sores begin as raised, round lesions in the mouth that turn into painful ulcers.
  • Skin: Skin lesions may occur in people with Behçet. Raised and tender nodules especially on the lower legs are observed.
  • Eyes: Behçet is likely to cause inflammation in the eye called as uveitis. It causes redness, pain and blurred vision in one or both eyes sporadically. Inflammation that occurs in the blood vessels of the retina is a serious complication of the disorder.
  • Joints: Joint swelling and pain often affect the knees in people with Behçet. The ankles, elbows or wrists also may be involved. Signs and symptoms may last one to three weeks and go away on their own.
  • Genitals: People with Behçet are susceptible to sores on their genitals in the form of red, ulcerated lesions.
  • Vascular System: Inflammation in veins and large arteries may occur, causing redness, pain and swelling in the arms or legs when blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.
  • Digestive System: Behçet may cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea or bleeding. Behçet can also lead to headache, fever, disorientation or stroke.

Causes

  • Behçet disease is a sporadic disease, but it is researched to be hereditary in nature.
  • Doctors have not delineated Behçet disease cause. However, a combination of genetic and environmental factors likely plays a role. Several genes have been found to be associated with the disease. Some researchers believe a virus or bacterium may cause Behçet in people with certain genes that make them susceptible to Behcet’s.

Diagnosis
No tests can determine definitively whether or not you have Behçet . Instead, your doctor relies primarily on your signs and symptoms to diagnose Behçet . Your doctor may conduct blood tests or other laboratory tests to rule out other diseases and conditions.

Criteria have been established for the diagnosis of Behçet , but these aren’t always essential for the diagnosis of the disease. Your doctor may use other factors for the diagnosis.

Mouth sores that have recurred at least three times in 12 months is a main diagnostic feature.

An ophthalmologist identifies signs of inflammation in eyes. A variety of rashes or acne-like sores may be caused by Behçet .

A pathergy test is performed wherein the doctor inserts a sterile needle into skin and then examines the area 1-2 days later. When pathergy test is positive, a small red bump is seen under skin at the spot where the needle was inserted.

Treatments
An exact cure does not exist for Behçet. In case of mild symptoms, the doctor may offer medications to control temporary flares in pain and inflammation. In case of severe signs, doctor may advise medications to control the signs and symptoms of Behçet throughout your body. Few criteria such as age and sex may influence the specific treatment recommended by the doctor.

Behçet may occur and fade on its own. Healthcare specialist may prescribe skin creams, gels and ointments. Topical medicines are used as direct application for sores to decrease inflammation and pain. Corticosteroid content in mouthwashes and eye drops help in combating inflammation.

When above medications fail to respond, Corticosteroids are administered to control the inflammation. Corticosteroids, such as prednisone, in combination with other medications may reduce the inflammation. Counter-effects such as weight gain, persistent heartburn, high blood pressure and bone thinning (osteoporosis) have been found to occur. Immunosuppressive drugs including azathioprine (Imuran, Azasan), cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide (Cytoxan) are used. These medications increase infection risk and likely cause liver and kidney problems, low blood counts, and high blood pressure.

Medications such as infliximab (Remicade) and etanercept (Enbrel blocks a substance called tumor necrosis factor (TNF) work against treatment of Behçet , especially for those with severe or resistant symptoms. Side effects may include headache, skin rash and an increased risk of upper respiratory infections.

Other drugs that have been used to treat Behçet include thalidomide (Thalomid), mycophenolate mofetil (CellCept) and chlorambucil (Leukeran).

References
1. Adamantiades B. A case of recurrent hypopyon iritis.Medical Society of Athens. 1930; 586-93.

2. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. International Study Group for Behçet’s Disease.Lancet. May 5 1990;335(8697):1078-80.

3. Emmi L, Brugnolo F, Salvati G, et al. Immunopathological aspects of Behçet’s disease.Clin Exp Rheumatol. Nov-Dec 1995;13(6):687-91.

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