Behçet syndrome

Behçet syndrome, also known as Behçet's disease, is a rare, chronic, autoimmune disease that causes blood vessel inflammation throughout the body. The disease is named after Hulusi Behçet, a Turkish dermatologist who first described the condition in 1937.

Symptoms

The symptoms of Behçet syndrome can vary widely among individuals but commonly include:

• Oral ulcers: Painful sores in the mouth.
• Genital ulcers: Sores on the genital area.
• Uveitis: Inflammation of the uvea in the eye, which can lead to blindness.
• Skin lesions: Various types of skin rashes and lesions.
• Arthritis: Joint pain and swelling.
• Gastrointestinal issues: Abdominal pain, diarrhea, and bleeding.
• Neurological symptoms: Headaches, meningitis, and other central nervous system issues.

Causes

The exact cause of Behçet syndrome is unknown. It is believed to be a combination of genetic and environmental factors. The presence of the HLA-B51 gene is strongly associated with the disease, particularly in individuals of Mediterranean, Middle Eastern, and East Asian descent.

Diagnosis

Diagnosis of Behçet syndrome is primarily clinical, based on the presence of characteristic symptoms. The pathergy test, which involves pricking the skin with a needle to see if a small red bump forms, can also aid in diagnosis. Other conditions that may present similarly, such as systemic lupus erythematosus, Crohn's disease, and herpes simplex, need to be ruled out.

Treatment

There is no cure for Behçet syndrome, but treatment focuses on reducing symptoms and preventing complications. Common treatments include:

• Corticosteroids: To reduce inflammation.
• Immunosuppressants: To control the immune system.
• Colchicine: Often used to treat arthritis and skin lesions.

Prognosis

The prognosis for Behçet syndrome varies. Some individuals may experience mild symptoms, while others may have severe complications such as blindness, thrombosis, and aneurysms. Early diagnosis and treatment are crucial for managing the disease effectively.

Related Pages

• Autoimmune disease
• Hulusi Behçet
• HLA-B51
• Uveitis
• Systemic lupus erythematosus
• Crohn's disease
• Herpes simplex