A Pemphigus is a skin disease in which watery blisters form under the skin.
clinical variants
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
pathogenesis
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]
etiologies
Autoantibodies against
desmoglein 3 and/or
desmoglein 1
epidemiology
N/A
risk factors
N/A
labimaging
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)
complications
side effects of steroids; high fatality w/o tx
screeningeducation
Pretty good prognosis with treatment
