Hulusi Behçet

Behçet's disease
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Oral ulcers, genital ulcers, uveitis, skin lesions
Complications	Blindness, thrombosis, aneurysms
Onset	Usually 20-30 years old
Duration	Chronic
Types	N/A
Causes	Unknown, possibly genetic and environmental factors
Risks	HLA-B51
Diagnosis	Clinical diagnosis, pathergy test
Differential diagnosis	N/A
Prevention	N/A
Treatment	Corticosteroids, immunosuppressants
Medication	N/A
Prognosis	N/A
Frequency	Rare
Deaths	N/A

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's disease vary from person to person and may include:

• Oral ulcers: Painful sores in the mouth, similar to canker sores.
• Genital ulcers: Sores on the genitals that can be painful and may scar.
• Uveitis: Inflammation of the uvea, the middle layer of the eye, which can lead to vision problems.
• Skin lesions: Various types of skin rashes or bumps, including erythema nodosum and pseudofolliculitis.
• Arthritis: Joint pain and swelling, often affecting the knees and ankles.
• Thrombophlebitis: Inflammation of veins, which can lead to blood clots.
• Gastrointestinal symptoms: Abdominal pain, diarrhea, and bleeding.
• Neurological symptoms: Headaches, confusion, and stroke-like symptoms.

Causes

The exact cause of Behçet's disease is unknown. It is believed to be an autoimmune disorder, where the body's immune system mistakenly attacks its own tissues. Genetic factors may play a role, as the disease is more common in people with the HLA-B51 gene. Environmental factors, such as infections, may also trigger the disease in genetically predisposed individuals.

Diagnosis

Diagnosing Behçet's disease can be challenging due to the variability of symptoms. There is no specific test for the disease. Diagnosis is primarily based on clinical criteria, which include recurrent oral ulcers along with at least two other symptoms such as genital ulcers, eye inflammation, or skin lesions. The pathergy test, where a small needle prick leads to an exaggerated skin reaction, may also support the diagnosis.

Treatment

There is no cure for Behçet's disease, but treatment can help manage symptoms and reduce the risk of complications. Treatment options include:

• Corticosteroids: To reduce inflammation and suppress the immune system.
• Immunosuppressants: Such as azathioprine, cyclophosphamide, and methotrexate to control the immune response.
• Biologic agents: Such as infliximab and adalimumab for severe cases.
• Colchicine: For joint and skin symptoms.

Prognosis

The prognosis for Behçet's disease varies. Some people experience mild symptoms, while others may have severe complications such as blindness or neurological damage. Early diagnosis and treatment can improve the outlook and quality of life for those affected.

See also

• Autoimmune disease
• Vasculitis
• Hulusi Behçet

External links

• Behçet's Disease Information
• NHS - Behçet's Disease