potentially fatal blistering disease
Autoimmune condition
bullae flaccid and surface removed easily
all races, esp. Ashkenazy jews, Indian subcontinent
can be drug induced (rare)
split within epidermis
immunofluorescence shows IgG surrounding epidermal cells
development autoantibodies against desmosome proteins
superficial intraepidermal split
IgG within the epidermis
Onset usually middle age
Mucosal involvement common
flaccid blisters
surface easily removed
particularly trunk
sore rather than itchy
Nikolsky sign
skin traction cause bulla to expand sideways
50% patients have oral involvement
very high dose prednisolone
azathioprine
cyclophosphamide
cyclosporin
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