What is the treatment for pemphigoid?

Treatment of Pemphigoid

Bullous Pemphigoid: First-Line Treatment

Superpotent topical corticosteroids (clobetasol propionate) are the first-line treatment for bullous pemphigoid, providing superior disease control with significantly lower mortality compared to systemic corticosteroids. 1

Initial Dosing Strategy by Disease Extent

• Localized/limited disease: Apply superpotent topical corticosteroids directly to lesions only 1

• Mild disease with widespread distribution: Apply to whole body except face 1

• Moderate disease: Start with clobetasol propionate 10-30 g per day 2

  • This mild regimen achieves 98% disease control and reduces cumulative corticosteroid exposure by 70% 2
  • In moderate BP, this approach nearly halves the risk of death or life-threatening adverse events compared to higher doses (hazard ratio 0.54) 2

• Extensive/severe disease: Apply clobetasol propionate 20 g per day (10 g per day if weight <45 kg) over entire body except face 1

  • Escalate to 40 g per day if disease control not achieved within 1-3 weeks 1
  • Achieves disease control in 73.5% of severe cases 3

Treatment Monitoring and Adjustment

• Assess response after 1-3 weeks: Disease control is defined as absence of new lesions or healing of established lesions 1

• Begin tapering after 15 days once disease control is achieved 1

• Monitor for adverse effects: Skin atrophy (occurs in 14.9%), purpura (5.4%), and infections 1, 3

Maintenance Phase

• After 4 months of treatment: Reduce to maintenance therapy of 10 g once weekly, applied preferentially to previously affected areas 1

• Continue maintenance for 8 months (total treatment duration 12 months) 1

• Follow-up schedule: Every 2 weeks for first 3 months, then monthly for next 3 months, then every 2 months 1

• Consider discontinuation after 12 months if symptom-free for at least 1-6 months on minimal therapy 1

Relapse Risk Assessment

• Positive direct immunofluorescence or anti-BP180 ELISA >27 U/mL indicates increased risk of relapse 4, 1

• Relapse definition: ≥3 new lesions per month or extension of established lesions 1

Second-Line Treatment Options for Bullous Pemphigoid

When topical corticosteroids fail, systemic therapy becomes necessary, but dosing must be carefully calibrated.

Oral Corticosteroids

• Start with oral prednisone 0.5 mg/kg/day for patients with moderate to severe disease 5, 1, 6

  • This dose achieves disease control in 62.6% of patients at day 21 6
  • Control rates vary by severity: 75% in mild BP, 68.8% in moderate BP, 46.4% in severe BP 6
  • One-year survival is 82.6% overall with this regimen 6

• For severe involvement: Consider 0.75-1 mg/kg/day 5

• Critical caveat: Doses >0.75 mg/kg/day do not confer additional benefit and are associated with significantly higher mortality 1

  • Higher doses (1.25 mg/kg/day) showed no statistical advantage over 0.75 mg/kg/day but caused more adverse effects 5

Adjunctive Immunosuppressants

• Azathioprine: Allows reduction of steroid dose by approximately 45% 1

  • Dose: 2-3 mg/kg/day (if TPMT normal) 5

• Mycophenolate mofetil: 2-3 g per day 5

Alternative Non-Immunosuppressive Options

• Tetracyclines combined with nicotinamide may be effective when combined with topical corticosteroids 1
  • Tetracycline 500-2000 mg daily, doxycycline 200-300 mg daily, or minocycline 100-200 mg daily 1
  • Avoid tetracycline in renal impairment and doxycycline/minocycline in hepatic impairment 1
  • Discontinue minocycline if hyperpigmentation, pneumonia, or eosinophilia develop 1

Osteoporosis Prophylaxis

• Implement prevention measures for corticosteroid-induced osteoporosis when using systemic corticosteroids 5, 1

Pemphigus Vulgaris: Treatment Approach

The treatment strategy for pemphigus vulgaris differs substantially from bullous pemphigoid due to its more aggressive nature.

First-Line Therapy

Oral prednisolone 1 mg/kg/day (or equivalent) combined with an adjuvant immunosuppressant is the standard first-line approach. 5

• For milder cases: Start with 0.5-1 mg/kg/day 5

• Increase in 50-100% increments every 5-7 days if blistering continues 5

• Consider pulsed intravenous corticosteroids if >1 mg/kg oral prednisolone required, or as initial treatment in severe disease followed by 1 mg/kg/day oral prednisolone 5

Adjuvant Immunosuppressants (Start Simultaneously)

These agents are more important for remission maintenance than induction due to delayed onset 5:

• Azathioprine: 2-3 mg/kg/day (if TPMT normal) 5

• Mycophenolate mofetil: 2-3 g per day 5

• Rituximab: Rheumatoid arthritis protocol (2 x 1 g infusions, 2 weeks apart) 5

  • Rituximab efficacy is higher when administered early in disease course 7
  • Should be considered as first-line treatment to reduce cumulative corticosteroid doses 7
  • Note: Currently approved by NHS England as third-line treatment; regulatory approval varies by country 5

Tapering Strategy

• Begin tapering once remission is induced and maintained: Absence of new blisters and healing of majority of lesions (skin and mucosal) 5

• Aim to reduce to 10 mg daily or less 5

Second-Line Therapy

• Switch to alternate corticosteroid-sparing agent if treatment failure with first-line adjuvant drug 5

• Mycophenolic acid 720-1080 mg twice daily if gastrointestinal symptoms from mycophenolate mofetil 5

Third-Line Therapy Options

Consider based on individual patient assessment 5:

• Cyclophosphamide
• Immunoadsorption
• Intravenous immunoglobulin
• Methotrexate
• Plasmapheresis or plasma exchange

Oral Mucosal Management

• Topical corticosteroid mouthwashes: Betamethasone sodium phosphate 0.5 mg dissolved in 10 mL water as 2-3 minute rinse-and-spit solution 1-4 times daily 5

• Fluticasone propionate nasules diluted in 10 mL water twice daily 5

• Clobetasol 0.05% ointment mixed in 50% Orabase applied twice weekly to localized lesions on dried mucosa 5

• Tacrolimus 0.1% ointment: In split-mouth trial, showed equivalent efficacy to triamcinolone acetonide 0.1% paste 5

Common Pitfalls to Avoid

• Do not use high-dose systemic corticosteroids as first-line for bullous pemphigoid: Topical therapy is safer and more effective 1

• Do not exceed 0.75 mg/kg/day oral prednisone for BP: Higher doses increase mortality without improving outcomes 1

• Do not delay adjuvant immunosuppressants in pemphigus vulgaris: Start simultaneously with corticosteroids, not sequentially 5

• Do not ignore osteoporosis prophylaxis: Assess risk immediately when starting systemic corticosteroids 5, 1

• Do not continue aggressive treatment for occasional blisters during maintenance: This represents acceptable disease control, not treatment failure 5