Introduction
Behcet's syndrome is a complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins. The precise aetiology has yet to be elucidated however. The classic triad of symptoms are oral ulcers, genital ulcers and anterior uveitis
Epidemiology
- Incidence: 1.00 cases per 100,000 person-years
- Peak incidence: 30-40 years
- Sex ratio: 1:1
| Condition | Relative incidence |
|---|---|
| Genital herpes | 300.00 |
| Behcet's syndrome | 1 |
| Takayasu's arteritis | 0.26 |
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Pathophysiology
Epidemiology
- more common in the eastern Mediterranean (e.g. Turkey)
- more common in men (complicated gender distribution which varies according to country. Overall, Behcet's is considered to be more common and more severe in men)
- tends to affect young adults (e.g. 20 - 40 years old)
- associated with HLA B51, a split antigen of HLA B5
- around 30% of patients have a positive family history
Clinical features
Features
- classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
- thrombophlebitis and deep vein thrombosis
- arthritis
- neurological involvement (e.g. aseptic meningitis)
- GI: abdo pain, diarrhoea, colitis
- erythema nodosum
Investigations
Diagnosis
- no definitive test
- diagnosis based on clinical findings
- positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)