Pemphigus Vulgaris - Highlights
Pemphigus vulgaris (PV) is an auto-immune disease characterized by intra-epithelial blister formation.
📝Here are some "Highlights" or a high level overview of Pemphigus Vulgaris
Antibodies, predominantly IgG4 subtype of the IgG antibody, attack a specific component of the desmosome called desmoglein (Dsg).
In PV Dsg3 is the main antigen attacked, but 50% patients also develop auto-antibodies to Dsg1.
A characteristic feature of PV is elicitation of the Nikolsky’s sign.
Conditions which show desquamative gingivitis include OLP, pemphigus vulgaris, cicatricial pemphigoid, epidermolysis bullosa and linear IgA disease.
Epithelium shows acantholysis, usually in the parabasal layers resulting in an intraepithelial-split. This is also called a supra-basilar split.
The basal cells remain attached to the basement membrane and resemble a “row of tombstones”.
Immunofluorescence positive for IgG and C3 --> chicken wire appearance.
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