Behcet's syndrome

[meme nony]

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
•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 B5* and MICA6 allele
•c. 30% of patients have a positive family history

Features
•classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
•thrombophlebitis
•arthritis
•neurological involvement (e.g. aseptic meningitis)
•GI: abdo pain, diarrhoea, colitis
•erythema nodosum, DVT

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)

*more specifically HLA B51, a split antigen of HLA B5