Autoantibodies inhibit adhesive fn of Dsg and lead to loss of cell-cell adhesion of keratinocytes causing blisters
IgG autoantobodies against Dsg1 and Dsg3 are pathogenic
and have 1ry role in blister formation
serum anti-Dsg1 and 3 autoantibodies measured by IIF or ELISA correlate with dz severity
IgG from PV sera w extracell domain of
Dsg3 causes suprabasilar acantholysis
when injected into neonatal mice
After immunoabsorption of
anti-Dsg3, serum no longer
produces blisters
Immunoabsorption of PF sera with extra cell domains of Dsg1 eliminates pathogenic activity.
IgG from patient's sera can cause superfine blisters in
neonatal mice
Monoclonal Abys against Dsg from a
mouse model of pemphigus and from
human patients induce blisters
histology similar to those in patients
with naturally occurring dz