Pemphigus vulgaris

defining characteristics

Inter-epidermal blisters, jagged & flaccid blisters w/ positive Nikolsky sign (easy to deform w/ slight pressure), hyperpigmentation in healing areas, not too much scarring, severe nail disease, oral lesions

disease development

Autoimmune blistering disorder where antibodies (IgG) are attacking intra-epidermal (suprabasal) proteins of desmosome, resulting in compromised cell-cell adhesion [basal layer splits from spinous layer]

potential causes

Autoantibodies against
desmoglein 3 and/or
desmoglein 1


risk factors


Histology? Basal epidermis still attached to BM, and rete ridges still visible but huge white space above represents blister (suprabasal split)
Chicken wire appearance w/ direct immunofluorescence

conventional treatment

Immunosuppressants, corticosteroids; new therapies (tyrosine kinase inhibitors to increase resistance to pemphigus IgG)


Side effects of steroids; high fatality w/o tx


Pretty good prognosis with treatment