behçets disease Flashcards
what
complex inflammatory condition
characteristically presenting with recurrent oral and genital ulers
link with what gene
HLA B51
prognostic indicator of severe disease
HLA B51
features: mouth ulcera
3 episodes per year
painful, circumscribes w red halo
heal over 2-4wks
features: genital ulcers
kissing ulcers
features: skin
erythema nodosum
papules + pustules
vasculitic type rashes
features: eyes
anterior or posterior uveitis
retinal vasculitis
retinal haemorrhage
features: MSK
AM stiffness
arthralgia
oligoarthritis usually knee or ankle
features: GI
inflammation affecting
- ileum
- caecum
- asc colon
features: CNA
memory impairment
headache
aseptic meningitis
meningoencephalitis
features: veins
budd chiari syndrome
DVT
thrombus in pulmonary veins
cerebral venous sinus thrombosis
features: lungs
pulmonary artery aneurysms can develop
if rupture can be fatal
diagnosis based on
clinicla features
Ix = pathergy test
pathergy test
sterile needle to create s/c abrasion on forearm
review it 24-48hrs after to look for a weal 5mm or more
tests for non-specific hypersensitivity of skin
when can pathergy test be positive
behcet’s disease
sweets syndrome
pyoderma gangrenosum
Mx
topical and systemic steroids colchicine topical anaesthetics immunosuppresion biologics
prognosis
relapsing remitting condition
normal life expectancy and can go into remission
inc mortality with haemoptysis, neurolgical involvement