Pemphigus Vulgaris Pathogenesis — MFDS Part 1 MCQ

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HardOral PathologyPemphigus Vulgaris PathogenesisMFDS Part 1

A patient has painful, persistent oral erosions without cutaneous lesions. Histopathology shows suprabasal acantholysis, and direct immunofluorescence demonstrates intercellular IgG throughout the epithelium. Which autoantigen is the principal target in this mucosal-dominant form of pemphigus vulgaris?

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Correct answer: B — Desmoglein 3

The correct answer is B, desmoglein 3. Pemphigus vulgaris is caused principally by IgG autoantibodies against desmosomal cadherins. In mucosal-dominant disease, anti-desmoglein 3 antibodies disrupt keratinocyte-to-keratinocyte adhesion, producing acantholysis and a suprabasal intra-epithelial split. Additional anti-desmoglein 1 antibodies are more characteristic of mucocutaneous disease. BP180, laminin-332 and integrin α6β4 are basement-membrane-zone targets associated with pemphigoid-spectrum subepithelial blistering. Type VII collagen is the principal target in epidermolysis bullosa acquisita, which also produces separation beneath rather than within the epithelium.

Reference: Rahimi S et al. Desmoglein-driven dynamic signaling in pemphigus vulgaris: a systematic review of pathogenic pathways. NPJ Regenerative Medicine. 2025;10:39. https://pubmed.ncbi.nlm.nih.gov/40846710/