Management of Bullous Pemphigoid
Clobetasol propionate 0.05% cream applied twice daily is the first-line treatment for bullous pemphigoid due to its effectiveness and fewer systemic side effects compared to oral corticosteroids. 1
Treatment Algorithm Based on Disease Severity
First-Line Therapy
Localized or Mild Disease:
- Topical therapy: Clobetasol propionate 0.05% cream applied to lesions twice daily 1
- If using systemic therapy: Prednisolone 20 mg or 0.3 mg/kg daily 2
Moderate Disease:
- Topical therapy: Clobetasol propionate 0.05% cream applied over the entire body (30-40g per day) in two daily applications, sparing the face 1
- If using systemic therapy: Prednisolone 40 mg or 0.6 mg/kg daily 2
Severe Disease:
- Topical therapy: Clobetasol propionate 0.05% cream (40g per day) 1
- If topical therapy is impractical or ineffective: Prednisolone 50-70 mg or 0.75-1 mg/kg daily 2
- Note: Higher doses (>0.75 mg/kg/day) do not provide additional benefit but increase toxicity 1
Important Considerations
- For elderly patients (>80 years), topical corticosteroids have shown the highest complete response rate (55%) with a low side-effect profile 3
- Implement osteoporosis prevention measures from the start of systemic corticosteroid therapy 2
Tapering Schedule
After achieving disease control (absence of new inflammatory or blistered lesions):
- Continue daily treatment for the first month
- Reduce to every 2 days in the second month
- Reduce to twice weekly in the third month
- Reduce to once weekly starting in the fourth month 1
For systemic corticosteroids:
- Begin tapering 15 days after disease control
- Reduce dose 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 1
Management of Inadequate Response
If inadequate response after 1-3 weeks:
- Increase clobetasol propionate up to 40g per day if patient is receiving less 1
- Consider adding steroid-sparing agents if disease control is not achieved within 3 weeks 1:
Refractory Disease Management
For cases not responding to conventional therapy:
- Rituximab has shown effectiveness in refractory cases (two 1000 mg IV infusions separated by 2 weeks, followed by 500 mg at month 12 and every 6 months thereafter) 1, 4
- Omalizumab has shown promise in some refractory cases 4
Monitoring and Prevention of Complications
Baseline Testing
- Complete blood count, renal and liver function, glucose, and infection screening 1
- Thiopurine methyltransferase activity before starting azathioprine 1
- G6PD levels before starting dapsone 1
Regular Monitoring
- Disease activity assessment every 1-2 months in stable patients 2
- Blood glucose monitoring for patients on systemic corticosteroids 1
- Electrolyte monitoring if diuretics are used 1
Prevention of Complications
- Proton pump inhibitors for gastric protection 1
- Calcium and vitamin D supplements 1
- Consider bisphosphonates to prevent osteoporosis 1
- Avoid furosemide due to its association with bullous pemphigoid 1
Wound Care
- Leave small to medium-sized blisters intact
- Drain larger blisters
- Apply non-adherent dressings to erosive lesions
- Consider antisepsis baths 1
Relapse Management
- 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
Clinical Pearls
- The aim of treatment is to suppress clinical signs sufficiently to make the disease tolerable, not complete suppression 2
- The occasional blister is acceptable and indicates the patient is not being over-treated 2
- Bullous pemphigoid is self-limiting and usually remits within 5 years 2
- In elderly patients, aggressive immunosuppression may pose greater risks than the disease itself 3