What is the treatment for Pemphigus?

Treatment of Pemphigus

Systemic corticosteroids are the first-line treatment for pemphigus, with adjuvant immunosuppressive agents added to reduce corticosteroid dose and maintain remission. 1

Diagnosis Confirmation

Before initiating treatment, confirm diagnosis with:

• Skin or mucosal biopsy for histology showing suprabasal acantholysis
• Direct immunofluorescence showing IgG deposition in intercellular spaces
• Indirect immunofluorescence or ELISA for anti-desmoglein antibodies 1

Treatment Algorithm

Phase 1: Remission Induction

1. Initial Therapy:

   • Oral prednisolone starting at 0.75-1 mg/kg/day (typically 40-60 mg daily for a 70 kg patient) 1
   • Higher doses do not confer additional benefit and increase mortality risk 1
   • Disease control typically takes several weeks to achieve (median 3 weeks) 1

2. Adjuvant Therapy (started simultaneously with corticosteroids):

   • Azathioprine (1-2.5 mg/kg/day) OR
   • Mycophenolate mofetil (2-3 g/day) 1
   • These reduce cumulative corticosteroid dose and associated side effects

3. For Severe or Recalcitrant Cases:

   • Rituximab has shown excellent efficacy (95% of patients achieve at least partial remission) 2, 3
   • Intravenous immunoglobulin (2 g/kg divided over 5 days) can provide rapid improvement while slower-acting drugs take effect 1

Phase 2: Maintenance Therapy

1. Corticosteroid Tapering:

   • Once disease is controlled (no new lesions, established lesions healing)
   • Reduce prednisolone dose gradually (e.g., by 5-10 mg every 2-4 weeks)
   • Accept occasional blisters during maintenance phase 1

2. Continued Adjuvant Therapy:

   • Continue azathioprine or mycophenolate mofetil
   • Aim for minimum effective doses of all medications

3. For Refractory Cases:

   • Cyclophosphamide (reserved for severe cases due to toxicity concerns)
   • Methotrexate (10-15 mg weekly)
   • Dapsone (as steroid-sparing agent) 1

Topical Management

• Topical corticosteroids for limited disease or as adjunct to systemic therapy
• Gentle cleansing of blisters with antimicrobial solutions
• Pierce blisters at base with sterile needle but do not deroof
• Monitor for and promptly treat infections 1

Special Considerations

Pregnancy

• Prednisolone is the safest systemic agent (90% inactivated by placenta)
• Avoid mycophenolate mofetil, methotrexate, and cyclophosphamide
• Azathioprine and IVIG can be used if necessary 1

Children

• Generally more favorable prognosis than adults
• Similar treatment approach with careful dose adjustment 1

Drug-Induced Pemphigus

• Thorough medication review to identify potential triggers
• Discontinuation of offending drug when possible 1

Monitoring

• Regular clinical assessment of disease activity
• Monitor for corticosteroid and immunosuppressant side effects
• Consider antibody titers (anti-desmoglein) to guide therapy decisions 1

Pitfalls and Caveats

• Infection is a major cause of mortality - vigilant monitoring is essential
• Pain control is critical for quality of life - address both background and procedural pain
• Corticosteroid-related complications can be severe - use minimum effective dose
• Avoid abrupt discontinuation of therapy as this may precipitate flares
• Rituximab is emerging as a potential first-line therapy due to superior outcomes and reduced corticosteroid requirements 4, 3

Pemphigus remains a challenging disease with significant morbidity and mortality. Early aggressive treatment with careful monitoring and appropriate adjuvant therapy offers the best chance for disease control and improved quality of life.