Bullous Disorders Flashcards
define bullous disorders
damage to adhesion mechanisms
diagnosis
Nikolsky’s sign
skin biopsy with direct immunofluorescence
indirect immunofluorescence
diagnosis fo pemphigoid
immunofluorescence shows linear IgG and complement deposited around the BM
management
systemic steroids
immunosuppression
emollients, topical steroids and antiseptics
management for pemphigoid
tetracyclines
define pemphigus
autoimmune condition with IgG autoantibodies to desmoglein 3 which maintains desmosome attachments
immune complexes form causing complement activation and protease release
end results is acantholysis
define acantholysis
loss of coherence between keratinocytes
buzzword for pemphigus
flaccid blisters that can burst easily to form erythematous erosions
where is pemphigus located?
scalp face axilla groin trunk mucosal surfaces e.g. mouth and respiratory tract
define pemphigoid
subepidermal blisters with no acantholysis
describe the immune reaction in pemphigoid
circulating IgG react with antigens on hemidesmosomes anchoring basal cells to the BM leading to complement activation and tissue damage
where do blisters form in skin layers in pemphigoid
basal layer and dermal papillae
buzzword for pemphigoid
large tense bullae that are itchy and cannot burst
three types of bullous disorders
pemphigus
pemphigoid
dermatitis herpetiformis
define dermatitis herpetiformis
AI bullous disease with strong association to Coeliac’s (HLA-DQ2)
