Treatment of Bullous Pemphigoid
Topical clobetasol propionate 0.05% cream applied twice daily to lesions is the first-line treatment for bullous pemphigoid due to its superior efficacy and safety profile compared to systemic corticosteroids. 1, 2
Treatment Algorithm Based on Disease Severity
First-Line Treatment
Mild to Moderate Disease:
Severe or Extensive Disease:
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 occurs within 4-17 days with topical clobetasol 3
Tapering Schedule
- For topical therapy: After complete healing plus 2 weeks, gradually switch to less potent topical corticosteroids 1, 3
- For systemic therapy:
- Begin tapering 15 days after disease control is achieved
- Reduce dose by one-third or one-quarter down to 15 mg daily at fortnightly intervals
- Then by 2.5 mg decrements down to 10 mg daily
- Finally by 1 mg each month
- Aim for minimal therapy dose of 0.1 mg/kg/day within 4-6 months 1
Second-Line and Adjunctive Treatments
Steroid-Sparing Agents (when first-line treatment is insufficient or contraindicated)
- Dapsone: Start at 50 mg/day, therapeutic range 50-200 mg/day 1
- Azathioprine: 1-2.5 mg/kg/day 1
- Tetracyclines: Doxycycline 200-300 mg daily with nicotinamide 1
- Mycophenolate mofetil: 0.5-1g twice daily 1
- Methotrexate: 5-15 mg weekly 1
Refractory Disease
- For cases not responding to standard therapies, rituximab may be considered (375 mg/m² weekly for 4 weeks) 5
- Case reports show complete remission with rituximab in refractory cases 5
Monitoring and Adverse Effects Management
Monitoring
- For topical therapy: Monitor for local side effects
- For systemic therapy:
- Weekly blood counts initially, then monthly once stable
- Liver function tests every 3 months
- Consider anti-BP180 IgG ELISA at days 0,60, and 150 to predict outcome 1
Prevention of Complications
- Osteoporosis prevention:
- Vitamin D and calcium supplements from start of therapy
- Consider bisphosphonates for treatment >3 months 1
- Infection prevention:
Wound Care
- Leave small to medium-sized blisters intact
- Drain larger blisters
- Apply non-adherent dressings to erosive lesions
- Consider antisepsis baths 1
Management of Relapse
- If relapse occurs during tapering: Return to the previous effective dose
- If relapse occurs after treatment discontinuation: Restart at appropriate dose based on severity 1
- Note: Risk of relapse is higher with topical corticosteroids (HR 0.85 for systemic vs. topical) but this is outweighed by the significantly lower mortality risk with topical treatment 6
Important Considerations and Pitfalls
Mortality risk: Recent evidence shows significantly higher mortality with systemic corticosteroids compared to topical treatment (HR 1.43), along with increased risk of major adverse cardiac events (HR 1.33) and infections (HR 1.33) 6
Common pitfalls:
- Overtreatment with systemic corticosteroids when topical therapy would suffice
- Inadequate monitoring for complications in elderly patients
- Failure to implement osteoporosis prevention measures
- Too rapid tapering of corticosteroids leading to relapse
- Avoiding furosemide due to its association with bullous pemphigoid 1
Treatment duration: Remember that bullous pemphigoid is typically self-limiting and usually remits within 5 years. The goal is to suppress clinical signs sufficiently to make the disease tolerable, not complete suppression 1