Pemphigus Vulgaris: Clinical Aspects and Treatments

ABSTRACT Pemphigus vulgaris is a rare, but potentially life-threatening (5%-30%) intraepidermal blistering disease with a mean onset age between 40 and 60 years. Disease onset typically begins in the oral mucosa prior to spreading to the skin. Classic pemphigus vulgaris is characterized by fragile flaccid blisters on normal or erythematous skin that have a tendency to break and form erosions. Diagnosis of pemphigus vulgaris can be supported by direct immunofluorescence detection of immunoglobulin G and C3 deposits on the surface of keratinocytes and immunoserology studies to detect anti-desmoglein 1/3 autoantibodies. Systemic corticosteroids are the mainstay of pemphigus vulgaris treatment and are often combined with other immunosuppressive adjuvants to improve remission rates. This review focuses on the clinical presentation, risk factors, and treatment options for pemphigus vulgaris. GENERAL PURPOSE To review the clinical presentation, diagnostic evaluation, and management approaches for pemphigus vulgaris. TARGET AUDIENCE This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and registered nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES After participating in this educational activity, the participant will: 1. Summarize the clinical manifestations associated with pemphigus vulgaris. 2. Evaluate risk factors associated with pemphigus vulgaris. 3. Explain evidence-based diagnostic and treatment options for pemphigus vulgaris.