Pemphigus Vulgaris and Paraneoplastic Pemphigus

Pemphigus vulgaris and paraneoplastic pemphigus are rare, chronic, life-threatening autoimmune mucocutaneous bullous conditions precipitated by IgG autoantibodies binding to desmoglein 1, desmoglein 3, and the plakin family of adhesion proteins. Oral lesions may be the only lesions or first to appear in these conditions, making it paramount that oral and maxillofacial surgeons are well-versed in diagnosis and treatment. Initial management almost always involves pain management, hydration, and tissue biopsy followed by corticosteroid therapy. Adjuvant therapy has greatly improved the safety and quality of life for these unfortunate patients.