Behcet’s disease is a systemic vasculitis of unknown aetiology.
It was classically described as a triad of:
- Uveitis
- Oral ulcers
- Genital ulcers
The criteria for clinical diagnosis of Behcet’s disease has now extended to:
- Recurrent oral ulcers (at least 3 times in a year)
- With any 2 of:
- Genital ulcers
- Positive pathergy test – formation of a pustule in response to pin-prick
- Eye involvement (such as anterior or posterior uveitis, retinal vasculitis)
- Skin lesions (Erythema nodosum, foliculitis, other ulcerations)
Other features of Behcet’s disease can include
- Arthritis
- Gastrointestinal ulceration
- Neurological involvement
- Thrombophebitis
- Aneurysms, especially aortic aneurysms
Behcet’s disease is most common in patients of Mediterranean, Middle Eastern and Japanese origin.
Males are more affected than females
It is associated with HLA B51.
Severe disease is managed with steroids and immunosupression.
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