## First-Line Treatment of Pemphigus Vulgaris **Key Point:** Systemic corticosteroids (prednisolone or methylprednisolone) are the gold standard and first-line agent for pemphigus vulgaris, regardless of disease severity or extent. ### Mechanism & Rationale Pemphigus vulgaris is an autoimmune blistering disorder mediated by IgG autoantibodies against desmoglein 3 (mucosal) and desmoglein 1 (mucocutaneous). Systemic corticosteroids suppress T-cell and B-cell function, reducing autoantibody production and providing rapid control of disease activity. ### Dosing Strategy 1. **Induction phase:** Prednisolone 0.5–1 mg/kg/day (typically 40–60 mg/day) for 4–6 weeks until lesions cease forming 2. **Consolidation phase:** Gradual tapering over 3–6 months 3. **Maintenance:** Lowest effective dose to prevent relapse **High-Yield:** Early, aggressive corticosteroid therapy prevents disease progression and reduces mortality from secondary infection and malnutrition. ### Steroid-Sparing Agents (Adjunctive) Once disease is controlled, steroid-sparing agents are added to permit dose reduction: | Agent | Role | Onset | Notes | |-------|------|-------|-------| | Azathioprine | First-line steroid-sparer | 6–8 weeks | Requires TPMT testing; monitor LFTs | | Mycophenolate mofetil | Alternative steroid-sparer | 4–6 weeks | Better tolerated; preferred in some centres | | Rituximab | Refractory disease | 2–4 weeks | Anti-CD20 monoclonal; increasingly used | | Dapsone | Mucocutaneous disease | 24–48 hours | Rapid onset but limited efficacy as monotherapy | **Clinical Pearl:** Topical corticosteroids and antimicrobial mouthwashes are used adjunctively for oral lesions, but systemic therapy is essential for disease control. **Warning:** Azathioprine and mycophenolate alone are NOT sufficient for initial management — they are too slow-acting and lack the immunosuppressive potency needed for acute disease.
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