Management of Bullous Pemphigoid
Topical clobetasol propionate 0.05% cream applied twice daily to lesions only should be the first-line treatment for bullous pemphigoid due to its superior efficacy and significantly lower mortality risk compared to systemic corticosteroids. 1, 2
Treatment Algorithm Based on Disease Severity
First-Line Treatment
Mild to Moderate Disease:
Severe or Widespread Disease:
- If topical treatment is impractical due to extensive involvement:
- Prednisolone 0.5 mg/kg/day for mild/moderate disease
- Prednisolone 0.75-1.0 mg/kg/day for severe disease 1
- Note: Recent evidence shows increased mortality, MACE, and infection risk with systemic corticosteroids compared to topical treatment (HR 1.43,95% CI 1.28-1.58) 2
- If topical treatment is impractical due to extensive involvement:
Disease Control Assessment
- Evaluate for disease control after 2-3 weeks of treatment 1
- Disease control defined as absence of new inflammatory or blistered lesions 1
- Complete epithelialization typically achieved within 4-17 days with topical clobetasol 3
Tapering Schedule
- For topical therapy: After complete healing plus 2 weeks, transition to less potent corticosteroid creams 1, 3
- For systemic therapy (if used):
Second-Line and Adjunctive Treatments
Steroid-Sparing Agents (when first-line treatment fails or is contraindicated)
- Dapsone: Start at 50 mg/day, therapeutic range 50-200 mg/day 1
- Azathioprine: 1-2.5 mg/kg/day 1
- Tetracycline (e.g., doxycycline 200-300mg daily) with nicotinamide 1, 4
- Mycophenolate mofetil: 0.5-1g twice daily 1
- Methotrexate: 5-15 mg weekly 1
Biological Therapies for Recalcitrant Cases
- Consider for severe recalcitrant disease after failure of conventional therapies 5
- Options include:
- Rituximab
- Omalizumab
- Dupilumab
- Clinical improvement achieved in 78% of refractory cases with biologics 5
Monitoring and Supportive Care
Monitoring
- Weekly blood counts initially, then monthly once stable 1
- Liver function tests every 3 months 1
- Consider anti-BP180 IgG ELISA at days 0,60, and 150 to predict outcome 1
Wound Care
- Leave small to medium-sized blisters intact
- Drain larger blisters
- Apply non-adherent dressings to erosive lesions
- Consider antisepsis baths 1
Prevention of Complications
- Proton pump inhibitors for gastric protection
- Calcium and vitamin D supplements
- Consider bisphosphonates for osteoporosis prevention if systemic corticosteroids used >3 months 1
- Vaccinate against influenza and pneumococci (avoid live vaccines) 1
- Avoid furosemide due to association with bullous pemphigoid 1
Management of Relapse
- If relapse occurs during tapering: Return to previous effective dose 1
- If relapse occurs after treatment discontinuation: Restart at appropriate dose based on severity 1
- Note: Systemic corticosteroids have lower relapse risk (HR 0.85,95% CI 0.77-0.94) but higher mortality risk compared to topical treatment 2
Treatment Duration
- Bullous pemphigoid is typically self-limiting, usually remitting within 5 years 1
- Treatment can be discontinued in patients symptom-free for 1-6 months on minimal therapy 1
- Maintenance with less potent topical corticosteroids may be needed for 5 weeks to 13 months 3
Important Caveats
- Elderly patients require closer monitoring due to increased risk of adverse effects 1
- Despite lower relapse rates with systemic corticosteroids, the significantly higher mortality risk favors topical treatment as first-line therapy 2, 6
- The goal of treatment is to make the disease tolerable, not complete suppression 1