What is the treatment for bullous pemphigoid?

Treatment of Bullous Pemphigoid

Superpotent topical corticosteroids (clobetasol propionate 0.05% cream) are the first-line treatment for bullous pemphigoid, as they are more effective and have fewer systemic side effects than oral corticosteroids. 1

Treatment Algorithm Based on Disease Severity

Localized/Limited Disease

• First choice: Clobetasol propionate 0.05% cream applied to lesions only, twice daily 2
• Apply 10-20g per day to affected areas 1
• Continue until disease control is achieved, then begin tapering

Extensive Disease

1. First choice: Clobetasol propionate 0.05% cream, 30-40g per day (20g if weight <45kg)

   • Apply over entire body including normal skin and lesions (sparing face)
   • Apply in two daily applications 2
   • Continue until disease control (no new lesions, established lesions healing)

2. Tapering schedule (after disease control is achieved):

   • Daily treatment for first month
   • Every 2 days in second month
   • Twice weekly in third month
   • Once weekly starting in fourth month 2

3. If inadequate response after 1-3 weeks:

   • Increase dose up to 40g per day if receiving less 2

Systemic Therapy Options

When to Use Systemic Therapy

• For patients who fail topical therapy
• For very extensive disease
• When topical application is impractical

Systemic Corticosteroids

• Dosing based on severity:
  • Mild/localized: 0.5 mg/kg/day prednisolone
  • Moderate: 0.3 mg/kg/day prednisolone
  • Severe: 0.75 mg/kg/day prednisolone 1
• Higher doses (>0.75 mg/kg/day) increase toxicity without additional benefit 1
• Begin tapering 15 days after disease control 1

Steroid-Sparing Agents

When long-term therapy is anticipated or to reduce steroid exposure:

1. First-line adjuvants:

   • Azathioprine (1-2.5 mg/kg/day) 1
   • Mycophenolate mofetil (0.5-1g twice daily) 1

2. Alternative options:

   • Tetracyclines (500-2000mg daily) with nicotinamide (500-2500mg daily) 2, 1
   • Doxycycline (200-300mg daily) 2
   • Methotrexate (5-15mg weekly) 1
   • Dapsone (50-200mg daily) 1

3. For refractory cases:

   • Rituximab (two 1000mg IV infusions 2 weeks apart) 1
   • Intravenous immunoglobulin 1

Monitoring and Assessment

• Disease control definition: Absence of new inflammatory or blistered lesions 1
• Assess response to treatment within 1-3 weeks 1
• Monitor for:
  • Local adverse effects: skin atrophy (14.9%), purpura (5.4%) 3
  • Systemic effects: adrenocortical insufficiency, deep vein thrombosis 3

Prevention of Treatment Complications

• Proton pump inhibitors for gastric protection
• Calcium and vitamin D supplementation
• Consider bisphosphonates for osteoporosis prevention 1
• Avoid furosemide due to association with bullous pemphigoid 1

Relapse Management

• If relapse occurs during tapering: Return to previous effective dose 1
• If relapse occurs after treatment completion: Reinstate topical corticosteroids at 10-30g/day depending on extent 1

Important Considerations

• Advanced age and comorbidities (neurological, cardiovascular, neoplastic) make management challenging 2
• Initial management of extensive disease may require hospitalization in some healthcare systems 2
• Perform baseline testing before initiating treatment: CBC, renal/liver function, glucose, infection screening 1
• Measure thiopurine methyltransferase (TPMT) activity before starting azathioprine 2
• Check G6PD levels before starting dapsone 2

The evidence strongly supports topical clobetasol propionate as the preferred initial treatment for bullous pemphigoid, with systemic therapy reserved for cases that fail to respond or when topical application is impractical.