Behçet’s Disease

Behçet’s disease, also known as Behçet’s syndrome, is a chronic inflammatory disorder that causes inflammation of blood vessels throughout the body. It is a very rare disease, primarily affecting people of Middle Eastern or East Asian descent.

Symptoms

Vasculitis can affect any part of the body, making the symptoms of Behçet’s disease quite diverse. However, a characteristic triad includes oral ulcers, genital ulcers, and uveitis. The disease follows an intermittent course, alternating between active flare-ups and remission periods, though the intensity of active phases generally decreases over time. The most common symptoms include:

• Oral ulcers: These can appear anywhere in the mouth, often in clusters. They are round or oval, varying in size and depth. They tend to be painful and resolve within a few days, though they frequently recur.
• Blisters and pustular lesions on the skin: These resemble acne and may also involve inflammation of hair follicles.
• Erythema nodosum: Painful, reddish or purplish nodules under the skin.
• Genital ulcers: These are larger and deeper than oral ulcers. They are often painful and may leave scars.
• Uveitis: Inflammation of the eye causing redness, pain, light sensitivity, and blurred vision.
• Joint pain and inflammation.
• Abdominal pain, diarrhea, and bleeding in cases where the digestive system is affected.
• Headache, confusion, lack of coordination, and neck stiffness if the inflammation reaches the brain or spinal cord.

Causes

The exact cause of Behçet’s disease is unknown, but there is a consensus on its immune-related nature: inflammation of the blood vessels occurs due to an abnormal immune response triggered by an external factor, possibly a bacterium or virus. It is also believed that certain individuals may be genetically predisposed to mount this immune response due to specific markers present in their blood.

Risk Factors

The likelihood of developing Behçet’s disease increases with the following factors:

• Age: Most commonly affects people between 20 and 30 years old.
• Geographic origin: Higher incidence in Middle Eastern and East Asian populations.
• Sex: The disease tends to be more severe in men.
• Family history: Genetic factors associated with the disease can be inherited.

Complications

With appropriate treatment, Behçet’s syndrome usually has a good prognosis, and its severity tends to decrease over time. However, depending on the organs affected, it can lead to severe complications:

• Partial or total blindness if recurrent uveitis is not controlled.
• Thrombosis or aneurysms: Vasculitis can lead to the formation of blood clots in arteries or the weakening of blood vessel walls, which may result in strokes, kidney damage, or pulmonary hemorrhage, depending on the affected vessel.
• Memory loss or personality changes if the disease affects the nervous system.

Prevention

Since the exact cause of Behçet’s disease remains unknown, it cannot be prevented. The only way to minimize symptoms and complications is through proper treatment.

Which Doctor Treats Behçet’s Disease?

Behçet’s disease is diagnosed and treated by pediatric rheumatology specialists.