Behçet’s disease is a rare, chronic inflammatory disorder characterized by recurrent symptoms affecting multiple parts of the body. It’s classified as an autoimmune condition and a type of vasculitis, meaning it involves inflammation of blood vessels. The exact cause is unknown, but it’s thought to involve a combination of genetic predisposition (e.g., HLA-B51 gene association), immune system dysregulation, and environmental triggers. It’s more common in regions along the ancient Silk Road, particularly in Turkey, the Middle East, and parts of Asia, with a prevalence of 10-400 per 100,000 in these areas compared to 1-10 per 100,000 globally.
Key Symptoms (Classic Triad)
Recurrent Oral Ulcers: Painful sores in the mouth, similar to canker sores, occurring at least three times in a year.
Genital Ulcers: Painful sores on the genitals, often leaving scars.
Eye Inflammation: Uveitis or retinal vasculitis, causing pain, blurred vision, or potential blindness if untreated.
Other Symptoms
Skin Lesions: Acne-like sores, erythema nodosum, or papulopustular lesions.
Joint Pain: Arthralgia or arthritis, typically in knees or ankles.
Vascular Issues: Thrombosis, aneurysms, or vasculitis affecting veins or arteries.
Gastrointestinal: Abdominal pain, diarrhea, or ulcers in the digestive tract.
Neurological: Headaches, meningitis, or stroke-like symptoms (neuro-Behçet’s).
Pathergy Reaction: Exaggerated skin response to minor trauma, like a needle prick.
Symptoms vary widely between patients and can flare up unpredictably, followed by periods of remission.
Diagnosis
No specific test confirms Behçet’s. Diagnosis relies on clinical criteria, such as the International Study Group (ISG) criteria or the International Criteria for Behçet’s Disease (ICBD), which score recurrent oral ulcers, genital ulcers, eye lesions, skin lesions, and pathergy. Doctors may use blood tests (e.g., ESR, CRP) to check inflammation, imaging for vascular or neurological involvement, or eye exams. Differential diagnosis includes Crohn’s disease, lupus, or herpes-related conditions.
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