What is the recommended treatment for bullous pemphigoid?

Treatment of Bullous Pemphigoid

The first-line treatment for bullous pemphigoid is clobetasol propionate 0.05% cream applied to lesions twice daily, which is more effective and has fewer systemic side effects than oral corticosteroids. 1

Initial Treatment Approach

Topical Corticosteroid Therapy

• For localized or mild disease:

  • Clobetasol propionate 0.05% cream applied to lesions only, twice daily 1

• For extensive disease:

  • Clobetasol propionate 0.05% cream applied over the entire body (including normal skin and lesions, sparing the face)
  • Dosage: 30-40g per day (20g if weight <45kg) in two daily applications 1
  • This approach has shown significantly better survival, disease control, and fewer severe complications compared to oral corticosteroids in patients with extensive disease 2

Systemic Corticosteroid Therapy

When topical therapy is impractical or ineffective:

• Moderate disease: Prednisolone 0.3 mg/kg/day 1
• Mild/localized disease: Prednisolone 0.5 mg/kg/day 1
• Severe disease: Prednisolone 0.75-1.0 mg/kg/day 1

Important note: Higher doses of prednisolone (>0.75 mg/kg/day) do not appear to provide additional benefit and may increase mortality risk 3, 1

Steroid-Sparing Agents

For patients requiring long-term treatment or experiencing side effects from corticosteroids:

• First-line steroid-sparing options:

  • Azathioprine (1-2.5 mg/kg/day) 1
    • Note: Measure thiopurine methyltransferase activity before starting 1
  • Tetracycline (e.g., doxycycline 200-300mg daily) with nicotinamide 1

• Alternative steroid-sparing options:

  • Mycophenolate mofetil (0.5-1g twice daily) 1
  • Methotrexate (5-15 mg weekly) 1
  • Dapsone (50-200 mg daily) 1
  • Chlorambucil (0.05-0.1 mg/kg/day) 1

Monitoring and Treatment Adjustment

Baseline Testing

• Complete blood count
• Renal and liver function tests
• Blood glucose
• Infection screening 1

Disease Activity Assessment

• Reassess after 2 weeks to determine if disease control is achieved
• Control is defined as absence of new inflammatory or blistered lesions (can be assessed after 3 weeks of treatment)
• For stable patients, assess disease activity every 1-2 months 1

Treatment Adjustment

• If disease is controlled: Begin tapering corticosteroids
• If relapse occurs during tapering: Reinstate previous effective dose
• If relapse occurs after treatment suspension: Reinstitute topical corticosteroids at 10-30g/day depending on relapse extent 1

Prevention of Complications

• Osteoporosis prevention: Implement from start of systemic corticosteroid therapy

  • Calcium and vitamin D supplements
  • Consider bisphosphonates 1

• Gastric protection: Proton pump inhibitors 1

• Wound care:

  • Elevation of legs
  • Compression stockings (if no open lesions)
  • Leave small to medium-sized blisters intact
  • Drain larger blisters
  • Apply non-adherent dressings to erosions
  • Consider antisepsis baths 1

Important Considerations

• Bullous pemphigoid is typically self-limiting and usually remits within 5 years 1
• Avoid furosemide due to its association with bullous pemphigoid as an adverse reaction 1
• Rituximab may be considered for refractory cases, with higher efficacy when administered early in disease course 4
• Plasma exchange plus prednisone has shown better disease control than prednisone alone in some studies, but evidence is mixed 3

Treatment Algorithm

1. Assess disease extent: Localized/mild vs. extensive/severe
2. Initial therapy:
   • Localized/mild: Topical clobetasol propionate 0.05% cream to lesions only
   • Extensive: Topical clobetasol propionate 0.05% cream to entire body or oral prednisolone if topical therapy impractical
3. Reassess at 2-3 weeks
4. If controlled: Begin tapering corticosteroids
5. If not controlled: Increase dose or add steroid-sparing agent
6. For long-term management: Consider steroid-sparing agents to minimize corticosteroid exposure