Recurrent fever and aphthous stomatitis, mostly. However, there is no regular periodicity of the symptoms, and episodes of fever may last for weeks.
Traditionally associated with Mediterranean or Eurasia but in UK mostly white Caucasian.
Other features –
- genital ulcers,
- iridocyclitis and optic neuritis, even blindness
- skin rashes (classically erythema nodosum or pustulosis),
- disabling vascular (thrombosis, superficial phlebitis) and central nervous system complications may occur.
BPSU study found 1 case every 2 weeks in the UK. Median age of onset was 6yrs – but diagnosis 11yrs!
Colchicine as regular preventive treatment, else immunosuppressive treatment. Topical steroids or short courses oral steroids.