Immunobullous disorders Flashcards
what diseases come under the immunobullous disorders
bullous pemphigoid
pemphigus
dermatitis herpetiformis
what is the primary feature of bullous disorders
blistering
What is the most common type of pemphigus
pemphigus vulgaris
what is the pathology behind pemphigus
IgG antibodies attack desmoglein 3 withIN the epidermis superficially causing an intraepidermal blister and acantholysis
what is acantholysis
lysis of intercellular adhesion sites
features of pemphigus
superficial erosions
mucosal involvement
Fragile fluid filled flaccid blisters
Nikolsky sign positive
what is Nikolsky’s sign
rubbing of the bullae causes the skin to break away and peel
management of pemphigus
topical steroids and anaesthetics
PO steroids
immunosuppressants
what is the pathology behind bullous pemphigoid
IgG antibodies attack hemidesmosomes at the DEJ creating a DEEP SUBepidermal blister (no evidence of acantholysis)
investigations for bullous disorders
biopsy and immunofluorescence
features of bullous pemphigoid
itchy tense bullae, erythematous base
more common in elderly
Mucosal sparing
Niksolsky sign negative
Management of bullous pemphigoid
localised BP: high potent topical steroids
generalised:
tetracyclines
systemic steroids and immunosuppressants
what GI condition is associated with dermatitis herpetiformis
Coeliac disease
pathology behind dermatitis herpetiformis
granular IgA deposits in dermal papillae
features of dermatitis herpetiformis
itchy vesicles
symmetrical
elbows, knees, sacrum, buttocks, scapulae, face, scalp