Vesiculobullous Disease Flashcards
immunological oral disease
LOCAL - aphthous ulcers, lichen planus, OFG (tend to fall under cell mediated immunity)
SYSTEMIC with local effects - erythema multiforme, pemphigus, pemphigoid, lupus erythematosis, systemic sclerosis, sjogren’s (tend to fall under antibody mediated immunity)
immunological skin disease
skin and oral / genital mucosa share many common antibodies & epitopes
many blistering skin conditions therefore also affect the mouth
auto antibody attack on skin components causes loss of cell-cell adhesion
‘split’ then forms in skin that fills with inflammatory exudate & forms a vesicle/blister
desmosome vs hemidesmosome
Desmosomes facilitate adhesion between adjacent epithelial cells, whereas hemidesmosomes, named for their ultrastructural resemblance to half a desmosome, mediate adhesion between basal cells of epithelial tissues and the substratum
direct v indirect immunofluorescence
direct = antibody mediated tissue disease; antibody bound to tissue; targeted in DIF
indirect = circulating antibody not yet bound to tissue, detected by immunofluorescence from a plasma sample, not always useful for diagnosis - often good for monitoring disease activity
erythema multiforme
spectrum disorder of immunogenic related skin & mucosa ulceration with variable orofacial involvement
acute onset M>F; skin target lesions & in mucosa is ulcers
aetiology = drugs, herpes simplex, mycoplasma
lips & anterior part of mouth - normally heals in 2wks but very painful; unable to eat / drink
stevens-johnson syndrome - severe multisystem involvement i.e. skin, conjunctivae, nose, pharynx, mouth, genitals
tx of erythema multiforme
for oral lesions:
urgent medical therapy - systemic steroids (up to 60mg/day i.e. high dose), systemic aciclovir
encourage fluid intake - may require admission for IV fluid if unable to drink
encourage analgesia
if recurrent - consider prophylactic aciclovir daily, allergy test, sometimes infective agent i.e. mycoplasma
angina bullosa haemorrhagica
commonest oral blistering condition
described as blood blisters in mouth
buccal mucosa & soft palate are common sites
rapid onset (a few mins)
last about 1hr then burst
relatively painless
possibly initiated by minor trauma
heal with no scarring within days
diagnosis of ABH
non specific ulceration
DIF, IIF negative
no platelet / coagulation defect
CHX mouthwash
may recur
management of ABH
no tx available at present
reassure pt that disease is benign
explain known triggers & course of disease
pemphigoid
relatively common
sub epithelial antibody attach
thick walled blisters (full epidermis) usually persist to be seen, clear or blood filled blisters
different forms & presentations of pemphigoid
bullous pemphigoid - skin
mucous membrane pemphigoid - all mucous membranes i.e. eye, genital, oral
cicatritial pemphigoid - mucosal with scarring
histopathology of pemphigoid
sub epithelial split - epithelial / connective tissue junction
hemi desmosomes involved at basement membrane
immunofluorescence in pemphigoid
linear staining along basement membrane
C3 and IgG detected in this area in ‘standard’ pemphigoid
IgA occasionally found
- linear staining with C3 is called liner IgA disease
- granular IgA and C3 deposits seen in dermatitis herpetiformis
pemphigoid signs & management
oral & skin lesions - both can cause lesions in both but bullous usually skin & mucous membranes usually mouth/eye/genital
scarring is a feature in some cases while healing
manage with immunosuppressants i.e. steroids / immune modulating drugs (azathioprine, mycophenolate)
pemphigus
commonest form is pemphigus vulgaris
intraepithelial blue
clinical - uncommon <50yrs, F»M
sites - skin, mucosa, both eventually involved
blisters burst then spread
rarely see intact bullae due to intra epithelial blister & surface easily lost
fatal without tx
