disease profiles Flashcards
autoimmune skin condition which antibodies are produced against hemi desmosomes proteins that are involved in the maintenance of the dermo epidermal junction
bullous pemphigoid
what does bullous pemphigoid result in
interuption of the dermo epidermal junction and the formation of sub epidermal blisters
presentation of bullous pemphigoid
elderly, well dermarcated plaques may present 1 year prior to blisters, large tense itchy blisters on skin or erythemous base typically trunk or proximal limbs
nikolsky sign what in bullous pemphigoid
negative
what is nikolski sign
skin finding in which the top layers of the skin slip away from the lower layers when rubbed
how to diagnose bullous pemphigoid
biopsy - sub epidermal blisters and inflammatoru infiltrates within blister and immunodluorescence - linerar IgG + complement along the basement membrane
management of bullous pemphigoid (local disease)
high potency topical steroids
management of bullous pemphigoid (systemic disease
oral steroids +/- tetracycline +/- antihostamine
why is an antihistamine used in bullous pemphigoid ?
for sedating and antu pruritic properties
why is tetracycline used in bullous pemphigoid
steroid sparing agents
how to treat bullous pemphigoid if there is no response ti antihistamine or tetracycline
immunosuppression
autoimmune condition in which there is antibodies produced against desmoglein 3
pemphigus vulgaris
what is desmoglein 3
one of the desmosome proteins involved in cell-cell adhesion
what does pemphigus vulgaris result in
intra epidermal blisters and acantholysis
what is acantholysis
seperatiion of indiduals keratinocytes
presentation of pemphigus vulgaris
middle aged, multiple painful, flaccid, fragile blisters and erosions of the skin and mucous membranes
pemphigous vulgaris nikolsky sign
positive
diagnosis of pemphigous vulgaris
biopsy intra epidermal blister with accumulation of inflammatory cells within the dermis and immunofluorescence - chicken wire depositation of IgG within the epidermis
management of local pemphigous vulgaris
topical steroids and topical anaesthetics
management of systemic pemphigous vulgaris
high dose steroids and immuno suppresion + /- rituximab
what immunosuppressant is used in the management of pemphigois vulgaris
mycophenolate, azathioprine, dapsone or cyclophosphamide
autoimmune skin condition caused by auto antibodies against TTG caues sub epidermal blisters in skin
dermatitis herpetiformis
what is TTG
the antibody implacated in ceoliac disease
presentation of dermititis herpetiformis
small intenesly itchy blisters on an erythemous swollen base