Immunobullous Disorders Flashcards
what three conditions come under immunobullous disorders
pempigus
bullous pemphigoid
dermatitis herpetiformis
who does pemphigus vulgaris present in
over 40s
what causes the immune response in pemphigus vulgaris
IgG autoantibodies against desmoglein 3
describe the histology of pemphigus vulgaris
acanthosis - increased epithelial thickness
loss of integrity of epidermal cell thickness
describe the presentation of pemphigus vulgaris
flaccid vesicles that rupture to leave red raw areas
mucosal involvement of the eyes and genitalia common
nikolsky sign positive
describe nikolsky sign
test when the upper layers of the skin are rubbed, the superficial layers rub away
what is the treatment for pemphigus vulgaris
prednisolone 40mg
what is the difference between pemphigus and bullous pemphigoid
pemphigus - superficial
pemphigoid - deep, below DEJ
who does bullous pemphigoid typically affect
elderly patients
which bullous condition has the best prognosis
bullous pemphigoid
describe the pathogenesis of bullous pemphigoid
IgG autoantibodies to basement membrane/DEJ
what is the presentation of bullous pemphigoid
large tensa bulla on base of normal skin
usually localised to trunk/limbs
itch preceding blisters
nikolsky sign negative
what is the management of bullous pemphigoid
very potent steroid to lesions
prednisolone and reduce dose after 4 weeks if no new lesions
dermatitis herpetiformis is linked with what disease
coeliac
what is the presentation of dermatitis herpetiformis
very itchy, burning blisters
located on elbows, scalp, shoulder and ankles
