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Vasculitis & polymyalgia
Behçet’s Disease
Also known as: Behçet’s syndrome
A rare inflammatory disease of blood vessels that causes recurrent mouth and genital ulcers and can affect the eyes, skin, joints and brain.
Video lesson
Animated explainer
What it is
Behçet’s disease is a variable-vessel vasculitis, meaning it can inflame arteries and veins of any size. It is most common along the historic Silk Road, from the Mediterranean to East Asia, and usually starts in young adults.
Symptoms
Painful mouth ulcers that return at least three times a year, genital ulcers, acne-like skin lesions, tender red skin nodules, eye inflammation with blurred vision, joint pain and, less often, blood clots or neurologic symptoms.
How it’s diagnosed
There is no single test. Diagnosis rests on the pattern of symptoms. An eye exam is essential, and a pathergy test (a skin reaction to a needle prick) supports the diagnosis in some populations.
Treatment
Colchicine and topical treatments help ulcers and skin disease, and apremilast is approved for mouth ulcers. Eye, vascular or neurologic involvement needs stronger immune-suppressing treatment such as azathioprine or TNF inhibitors.
Living with it
Regular eye checks are vital. Good oral hygiene and avoiding mouth irritants reduce ulcer flares.
At a glance
Category
Vasculitis & polymyalgia
Also known as
Behçet’s syndrome
Reviewed by Farhan Tahir, MD, FACR
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