Pemphigus Vulgaris (oral lesions)

Diagnosis: Pemphigus Vulgaris (oral lesions)

Erosions on the lips and desquamative gingivitis.

Clinical Presentation

Erosions on the lips and desquamative gingivitis.

Clinical History

Submitted by Alaa Saad. Originally posted November 25, 2010.

Treatment

See case discussion.

Differential Diagnosis

• Bullous pemphigoid • Paraneoplastic pemphigus • Linear IgA bullous dermatosis • Mucous membrane pemphigoid • Erythema multiforme major • Herpetic gingivostomatitis • Aphthous stomatitis

Key Learnings

• IgG autoantibodies against desmoglein 3 (mucosal) and desmoglein 1 (cutaneous) — targets of desmosomal adhesion • Nikolsky sign positive — lateral pressure causes epidermal detachment • Oral involvement is often the first and sometimes the only manifestation • Flaccid blisters that rupture easily (in contrast to tense bullae of BP) • DIF shows "chicken wire" or "fishnet" pattern of IgG and C3 in intercellular spaces • Desmoglein compensation theory explains mucosal vs. mucocutaneous phenotypes • Historically fatal — mortality dramatically reduced with rituximab and corticosteroids • Rituximab now considered first-line per RITUX 3 trial

Tags: pemphigus, vulgaris, oral, lesions, alaa saad