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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