What is the treatment for bullous pemphigoid?

Treatment of Bullous Pemphigoid

Very potent topical corticosteroids such as clobetasol propionate 0.05% cream should be used as first-line treatment for bullous pemphigoid, as they provide effective disease control with significantly lower mortality risk compared to systemic corticosteroids. 1, 2

First-Line Treatment Options

Topical Corticosteroids

• Very potent topical corticosteroids (clobetasol propionate 0.05% cream)
  • Apply 40g/day to affected and non-affected skin 1
  • Demonstrated superior survival and disease control with fewer severe complications compared to oral prednisone, especially in extensive disease 3
  • Associated with significantly lower risk of death (HR 1.43), major adverse cardiac events (HR 1.33), and infections (HR 1.33) compared to systemic corticosteroids 2
  • May have slightly higher risk of relapse compared to systemic treatment 2

Alternative First-Line Options

• Tetracyclines with nicotinamide
  • Particularly suitable for elderly patients with multiple comorbidities 1
  • Tetracycline 500-2000 mg daily, doxycycline 200-300 mg daily, or minocycline 100-200 mg daily, combined with nicotinamide 500-2500 mg daily 4, 1
  • Monitor for drug interactions and assess cardiac, renal, and hepatic function 1

Second-Line Treatment Options

Systemic Corticosteroids

• Prednisolone dosing based on disease severity:

  • Mild or localized disease: 0.5 mg/kg/day 4
  • Moderate disease: 0.3 mg/kg/day 4
  • Severe disease: 0.75-1.0 mg/kg/day 4
  • Higher doses (>0.75 mg/kg/day) do not provide additional benefit but increase toxicity 4, 5

• Tapering schedule:

  • Begin tapering 15 days after disease control is achieved 1, 5
  • Reduce by one-third or one-quarter down to 15 mg daily at fortnightly intervals
  • Then reduce by 2.5 mg decrements to 10 mg daily
  • Finally reduce by 1 mg each month 4

Steroid-Sparing Agents

• Azathioprine (1-2.5 mg/kg/day)

  • Allows reduction of prednisolone dose by almost half 3
  • Consider for patients who relapse on steroid tapering 1

• Mycophenolate mofetil (0.5-1g twice daily) 1

• Methotrexate (5-15 mg weekly) 1

• Dapsone (50-200 mg daily) 1

Treatment for Refractory Cases

• Rituximab

  • Two 1000 mg IV infusions separated by 2 weeks, followed by 500 mg at month 12 and every 6 months thereafter 1

• Intravenous immunoglobulin, cyclophosphamide, or plasmapheresis

  • Reserved for exceptional refractory cases 1

Disease Assessment and Monitoring

• Assess disease control after 3 weeks of treatment 1, 5
• Control is defined as absence of new inflammatory or blistered lesions 4
• A BPDAI score threshold of 49 points can help predict disease control at day 21 5
• Karnofsky score of 70 points correlates with 1-year survival 5

Prevention of Complications

• Use proton pump inhibitors for gastric protection 1
• Provide calcium and vitamin D supplements 1
• Consider bisphosphonates to prevent osteoporosis 1
• Avoid furosemide due to its association with bullous pemphigoid 1

Management of Relapse

• If relapse occurs during tapering, reinstate the previous effective dose 1
• If relapse occurs after treatment suspension, reinstitute topical corticosteroids at 10-30g/day depending on extent of relapse 1

Wound Care

• Elevate legs and use compression stockings if no open lesions are present 1
• Leave small to medium-sized blisters intact; drain larger blisters 1
• Apply non-adherent dressings to erosive lesions 1
• Use antiseptic baths for affected areas 1

The evidence strongly supports topical corticosteroids as first-line treatment due to their efficacy and safety profile, particularly in reducing mortality compared to systemic corticosteroids. Treatment should be tailored based on disease severity, with systemic steroids reserved for cases that don't respond adequately to topical therapy.