Bullous disorders Flashcards
what are they?
autoimmune diseases where there is damage to the adhesion mechanisms in the skin resulting in blistering at various levels
what is the general prognosis?
chronic self-limiting course, duration varies from months to years - most patients achieve remission on treatment within 3-6 months
what investigations can you do?
skin biopsy with direct immunoflorescence
indirect immunofluorescence
what is Nikolsky’s sign?
when the top layers of skin slip away from the lower layers when slightly rubbed - indicated a plane of cleavage within the dermis
What is pemphigus vulgaris?
a split which is more Superficial - intra epidermal
What areas are most affected by pemphigus vulgaris?
scalp face axillae groins mucosal involvement very common e.g. eyes and genitals
how does pemphigus vulgaris present?
flaccid vesicles/ bullae which are thin-roofed
lesions can rupture and leave raw areas - increased risk of infection
acantholysis - loss of intercellular connections causing cohesion loss between keratinocytes
poSitive Nikolsky’s sign
How is pemphigus vulgaris treated?
responds to steroids
if untreated the mortality is very high
what is bullous pemphigoid?
when the split is deeper through the DEJ - dermo-epidermal junction
who gets bullous pemphigoid?
elderly people
where do people get bullous pemphigoid?
can be localised or widespread on trunk and proximal limbs
how does bullous pemphigoid present?
large tense bullae on normal skin on erythematous base
may present with itchy erythematous plaques and papules
blisters burst and leave erosions
non-scarring
mucosal lesions unlikely
negative Nikolsky’s sign
what is the prognosis of bullous pemphigoid?
much lower risk of mortality that pemphigus vulgaris
What is dermatitis herpetiformis?
a condition linked to coeliac disease - autoimmune
how does dermatitis herpetiformis present?
papillary dermal microabsesses - halmark (90% of lesions have gluten sensitive enteropathy)
very itchy
scratching removes vesicles leaving behind erosions
