What is Behcet's Disease?
Behçet's disease is an inflammatory condition causing recurrent mouth ulcers, genital ulcers, skin lesions and eye inflammation, and it can also affect blood vessels, joints, the gut and the nervous system. Eye involvement is the most important complication, because untreated it threatens sight.
Also called: Behçet's syndrome, Silk Road disease
Who gets Behcet's Disease?
It is most common along the historic Silk Road — Turkey, Iran, the Middle East, Central Asia, China and Japan — and occurs in India, though less frequently. Onset is usually in the twenties and thirties. Disease tends to be more severe in men and in those with younger onset. HLA-B51 is associated with it but is neither necessary nor sufficient for the diagnosis.
What are the symptoms of Behcet's Disease?
Not everyone has every symptom below, and having one does not confirm the diagnosis. These are the features that most often lead to assessment:
- Painful mouth ulcers recurring at least three times a year — present in almost every patient and usually the first symptom
- Painful genital ulcers, often leaving scars
- Red painful eyes, blurring, floaters or light sensitivity — uveitis, which can threaten sight
- Acne-like spots or tender red nodules on the legs
- A pustule forming at a needle prick site (pathergy)
- Joint pain and swelling, usually without damage
- Deep vein thrombosis, sometimes in unusual sites
- Abdominal pain, diarrhoea or bleeding from gut ulceration
- Headache, weakness or confusion where the nervous system is involved
How is Behcet's Disease diagnosed?
There is no confirmatory blood test. The diagnosis is clinical, based on recurrent oral ulceration together with genital ulcers, eye inflammation, characteristic skin lesions or a positive pathergy test.
All patients need formal ophthalmology assessment, since eye disease can be present with minimal symptoms and is the main threat to long-term function.
Other causes of recurrent ulceration are excluded: iron, folate and B12 deficiency, coeliac disease, inflammatory bowel disease and infection.
Imaging is directed by symptoms — vascular studies for suspected clots, MRI for neurological involvement, endoscopy for gut symptoms.
How is Behcet's Disease treated?
Treatment is matched to which systems are involved, and eye and neurological disease are treated most aggressively.
Mucocutaneous disease: topical steroids, colchicine, and apremilast for resistant oral ulceration.
Eye disease: prompt systemic treatment with corticosteroids plus azathioprine or ciclosporin, and infliximab or interferon for sight-threatening disease. This is urgent, not elective.
Vascular disease: immunosuppression is the primary treatment, since the clots are inflammatory rather than simply thrombotic; anticoagulation alone is insufficient.
Neurological and gut involvement: corticosteroids with azathioprine or a TNF inhibitor.
Living with Behcet's Disease
The disease typically follows a relapsing and remitting course and often becomes less active with age. Regular ophthalmology review is essential even during quiet periods. Patients learn to recognise their own early warning signs, and treating a flare early is considerably easier than treating an established one.
When should you see a rheumatologist?
Recurrent mouth ulcers three or more times a year, particularly with genital ulcers, eye symptoms or unusual skin lesions, should be assessed. Any eye redness, pain or visual change needs same-week ophthalmology review.
Common questions about Behcet's Disease
Are recurrent mouth ulcers enough to diagnose Behçet's?
No. Recurrent mouth ulcers are common and usually have other causes — iron, B12 or folate deficiency, coeliac disease, or simple aphthous ulceration. The diagnosis requires additional features such as genital ulcers, eye inflammation or characteristic skin lesions.
Will it affect my eyes?
Eye involvement occurs in a substantial proportion of patients and is the main threat to long-term function. Everyone diagnosed should have a formal eye assessment, and any eye symptom needs prompt review, because sight-threatening inflammation can be present with only mild symptoms.
Does it get better with age?
For many patients, yes. Disease activity commonly declines over the years, particularly after the first decade. Monitoring continues because flares can still occur.
This page is general information to support a consultation. It is not a diagnosis, and it does not replace an assessment by a doctor who has examined you.