Pemphigus Vulgaris Flashcards
dg
skin biopsy,
- rounded-up separated keratinocytes (called acantholytic cells) within the blisters just above the basal layer of the epidermis
- tombstone appearance of granular layer d/2 sep
immunofluorescence staining of the skin biopsy sections to reveal antibodies
-+ve nikolsky
scoring of severityy
Pemphigus Disease Area Index
sx
initial oral mucosal blistering followed by skin
painful
slow healing
hoarse vouce w/ laryngeal involvement
describe blister
thin walled w/ clear fluid
superficial
vegetative erosions in kin folds called PEMPHIGUS VEGETANS
epidemiology
most commonly between the ages of 50-60 years, and is more common in Jews andIndians presumably for genetic reasons
rx
bno cure -reduce blisters -prevent 2ndary inf - Oral corticosteroids are the mainstay of medical treatment for controlling the disease
other immuno suppressives
- rituximab
- cyclophosphamide
- dapsone
minimixe skin trauma
- spicy had or acisic food
- contact sport
def
autoimmune disease characterized by superficial blisters in skin and mucous membranes
etio
(IgG) autoantibodies bind to a protein called desmoglein 3, which is found in desmosomes in the keratinocytes near the bottom of the epidermis. The result is the keratinocytes separate from each other, and are replaced by fluid, the blister.