Pemphigus Vulgaris
Pronunciation: PEM-fih-gus vul-GAR-is
A serious autoimmune blistering disorder in which IgG autoantibodies target desmoglein-3 (and sometimes desmoglein-1), causing intraepidermal blister formation in skin and mucous membranes.
Full Definition
Pemphigus vulgaris (PV) is a potentially life-threatening autoimmune condition characterised by flaccid bullae and erosions affecting the skin and oral mucosa. Pathogenic IgG antibodies disrupt desmoglein-mediated keratinocyte adhesion (acantholysis), leading to suprabasal cleft formation. It is distinguished from bullous pemphigoid by lesion morphology (flaccid vs. tense bullae), histological level of blister formation (intraepidermal vs. subepidermal), and target antigen. Editors must ensure precise use of 'pemphigus' versus 'pemphigoid', as these are distinct diagnostic categories with different management, and conflation constitutes a significant clinical error in any document.
Usage
Usage note: Never abbreviate to 'pemphigus' alone in clinical documents without disambiguation, as multiple pemphigus subtypes exist. Do not confuse with 'pemphigoid'.
In Context
- "First-line treatment for pemphigus vulgaris consists of systemic corticosteroids combined with rituximab or azathioprine." — Clinical guidelines
- "The editor flagged 'pemphigoid' in the abstract where the correct diagnosis was 'pemphigus vulgaris'—a critical error with treatment implications." — Peer-review editorial flag