How would you manage severe bullous pemphigoid with elevated erythrocyte sedimentation rate (ESR)?

Management of Severe Bullous Pemphigoid with Elevated ESR

For severe bullous pemphigoid with elevated ESR (75), systemic corticosteroids at a dose of 0.75-1 mg/kg/day of prednisolone should be initiated as first-line therapy, with careful consideration of steroid-sparing agents to minimize long-term complications. 1

Initial Treatment Approach

Systemic Corticosteroids

• Dosing for severe disease:
  • Prednisolone 0.75-1 mg/kg/day (not exceeding 1 mg/kg/day as higher doses don't provide additional benefit but increase toxicity) 1
  • The elevated ESR (75) indicates significant inflammation, supporting the need for adequate immunosuppression
  • Continue this dose until cessation of new blister formation (typically 1-4 weeks)

Monitoring Response

• Assess for control of disease within 1-3 weeks
• If inadequate response (continued formation of new blisters):
  • Consider increasing dose to maximum of 1 mg/kg/day if started at lower dose
  • Do NOT exceed this dose as studies show no additional benefit but significantly increased toxicity 1

Adjunctive Therapies

Steroid-Sparing Agents (to be initiated early)

• Azathioprine: 2-3 mg/kg/day (after checking TPMT levels) 1

  • Allows for approximately 45% reduction in total steroid dose 1
  • Particularly important given elevated ESR indicating high inflammatory burden

• Alternative options if azathioprine is contraindicated:

  • Mycophenolate mofetil: 2-3 g/day
  • Dapsone: Consider as adjuvant therapy (has shown 92% complete remission when added to existing regimen) 2

Topical Therapy

• Clobetasol propionate 0.05% cream/ointment applied to lesions
• For localized areas of involvement while systemic therapy addresses widespread disease 1

Tapering Protocol

Once disease control is achieved (no new blisters for 1-2 weeks):

1. Reduce prednisolone by approximately one-quarter to one-third of dose every 2 weeks until reaching 15 mg daily
2. Then reduce by 2.5 mg decrements to 10 mg daily
3. Finally, reduce by 1 mg each month thereafter 1
4. Be prepared for relapse in approximately 50% of cases during tapering 1

Special Considerations

Osteoporosis Prevention

• Initiate calcium and vitamin D supplementation at start of treatment
• Consider bisphosphonates for patients at high risk of osteoporosis
• Bone density monitoring as appropriate

Infection Risk

• The elevated ESR may indicate inflammatory burden or potential infection
• Monitor for signs of infection during immunosuppressive therapy
• Consider prophylactic antibiotics if high-risk

Refractory Disease Management

For patients who fail to respond to standard therapy:

• Rituximab: Consider for refractory cases (though associated with infusion reactions in 58% of patients) 3
• Intravenous immunoglobulin: May be beneficial as adjunctive therapy in difficult cases 4
• Plasma exchange: May allow for lower steroid doses in severe disease 1

Pitfalls to Avoid

1. Excessive steroid dosing: Doses of prednisolone >1 mg/kg/day do not improve outcomes but significantly increase mortality 1

2. Inadequate steroid-sparing strategy: Failure to introduce steroid-sparing agents early leads to prolonged high-dose steroid exposure and complications

3. Too-rapid tapering: Can lead to disease flares; follow the structured tapering schedule

4. Overlooking comorbidities: Elderly BP patients often have multiple comorbidities that can be exacerbated by treatment

5. Ignoring elevated ESR: The elevated ESR (75) indicates significant inflammation and possibly increased disease severity, requiring close monitoring and potentially more aggressive initial therapy

Remember that mortality during the first year is significantly higher in patients treated with high doses of systemic corticosteroids (prednisolone equivalent >40 mg daily), so balancing disease control with minimizing treatment-related complications is essential 1.