Behçet Disease Health Article

Definition

Behçet disease (BD), also known as Behçet syndrome, is a chronic form of vasculitis (inflammation of the blood vessels) involving four primary symptoms: oral and genital ulcers, ocular inflammation, and arthritis.

Description

Behçet disease was first described in the 1930s by Turkish dermatologist Hulusi Behçet. His observations of the three classic symptoms (oral and genital ulcers and eye inflammation) now define this complex condition. BD also has a unique ability to affect all sizes of blood vessels, including arteries and veins. Symptoms related to vasculitis, such as inflammation of joints, gastrointestinal areas, or the central nervous system, are also common.

Demographics

Incidence of BD is very rare in the United States with approximately five in 100,000 people developing the syndrome. In Middle Eastern and Asian countries between Iran and Japan (known as the "Old Silk Route"), BD is quite prevalent. Incidence in these countries is double that of the United States.

More than twice as many females are diagnosed with BD than males in the United States. However, in Middle Eastern and Asian areas, significantly more men are affected than females.

Causes and symptoms

Behçet disease is caused by an autoimmune response that triggers inflammation of the blood vessels. Researchers have discovered a gene, HLA-B51, which predisposes an individual to BD. However, not all individuals with this gene develop the disease. The specific event leading to onset of BD is not known, but there are speculations that it may be related to the following:

• herpes simplex virus infections
• frequent infections of Streptococcus bacteria
• environmental factors

The four primary symptoms of BD are recurring complications that rarely present simultaneously. These include:

• Oral ulcers (aphthous ulcers). Usually the first sign of disease, these sores resemble common canker sores, but are present in greater number, larger size, and occur more frequently. They may be painful and persist for up to two weeks.
• Genital ulcers. Similar in appearance to oral ulcers, genital sores typically occur on the scrotum in males and in the vulva in females. These ulcers are painful.
• Ocular inflammation (uveitis). May affect the front of or behind the eye, or both together. Inflammation of the middle eye area leads to blurred vision, light sensitivity, and possibly loss of sight.
• Arthritis. Temporary inflammation of the joints develops intermittently.

A large number of secondary symptoms are also associated with BD. These affect the following areas:

• Skin. Acne-like outbreaks of red skin sores develop on the legs and parts of the upper body.
• Vascular system. Formation of blood clots may lead to aneurysms or inflammation of veins (thrombosis). This is more frequent in men.
• Gastrointestinal system. Less often, patients may develop ulcers along the digestive tract.
• Central nervous system. Inflammation of the blood vessels in the brain can result in a variety of conditions such as headache, confusion, stroke, or seizures.

Diagnosis

Behçet disease is diagnosed based on a set of guidelines established by an international group of physicians. A physician observes clinical signs and symptoms during patient examination. The most recent and accepted guidelines for a positive diagnosis include the presence of recurring oral ulcers (three or more times in one year) and at least two of four secondary symptoms, including recurring genital ulcers, uveitis, skin lesions, a positive pathergy test.

A pathergy test is a skin-prick test to see if a red bump will form at the injection site. If there is a reaction, the test is positive. This test may be given to patients suspected of BD, but it is not an indicator for the disease. Only a small percentage of patients diagnosed with BD actually test positive.

Treatment team

Patients diagnosed with Behçet disease require a diverse treatment team due to the variety of symptoms and complications. The primary specialist is usually a physician who specializes in arthritis (rheumatologist). In addition, the team includes a dermatologist (skin), an ophthalmologist (eyes), a gynecologist or urologist (genital), a gastroenterologist (digestive system), and a neurologist (nervous system).