Behçet Syndrome

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
• around 30% of patients have a positive family history

Look for an Asian or Middle Eastern person with painful oral and genital ulcers in association with erythema nodosum-like lesions of the skin.

Also with:

1. Ocular lesions leading to uveitis and blindness (hence, most feared complication)
   • Conjunctivitis is seen rarely and is much less common than anterior uveitis.
   • Other ocular problems seen include retinal vasculitis, iridocyclitis and chorioretinitis
2. Pathergy - sterile skin pustules from minor trauma like a needle stick
3. Arthritis
4. GI: abdo pain, diarrhoea, colitis
5. CNS lesions mimicking multiple sclerosis; aseptic meningitis

(Recurrent) aphthous/mouth ulcers, genital ulcers, thrombophlebitis, deep vein thrombosis and aseptic meningitis are all recognised features of Behcet's syndrome

• it may follow viral infections, but etiology is unclear
• due to immune complex vasculitis involving small vessels

Diagnosis