What is the treatment for bullous pemphigoid?

Treatment for Bullous Pemphigoid

Superpotent topical corticosteroids (clobetasol propionate) are the first-line treatment for bullous pemphigoid, as they provide better disease control with significantly lower mortality compared to systemic corticosteroids. 1, 2

Initial Treatment Approach

For Localized/Limited Disease:

• Apply superpotent topical corticosteroids (clobetasol propionate) directly to lesions only 1
• For mild disease with more widespread distribution, apply to the whole body except the face 1

For Generalized Disease:

• Apply clobetasol propionate 20g per day (10g per day if weight <45kg) over the entire body except the face 1
• If disease control is not achieved within 1-3 weeks, increase to 40g per day 1
• Topical treatment has been shown to provide better survival rates and fewer complications compared to systemic steroids, particularly in extensive disease 2, 3

Treatment Monitoring and Adjustment

• Assess response after 1-3 weeks of initial treatment 1
• Once disease control is achieved (reduction of blisters, urticarial lesions, and pruritus), begin tapering after 15 days 1
• Disease control is defined as absence of new lesions or established lesions healing 1
• Monitor for skin atrophy, purpura, and infections as potential side effects of topical steroids 1

Maintenance and Tapering

• After 4 months of treatment, reduce to maintenance therapy of 10g once weekly, preferentially applied to previously affected areas 1
• Continue maintenance treatment for 8 months (total treatment duration of 12 months) 1
• Monitor for relapse, defined as ≥3 new lesions/month or extension of established lesions 1

Second-Line Treatment Options

If topical corticosteroids fail to control the disease:

• Oral prednisone at 0.5 mg/kg/day (doses higher than 0.75 mg/kg/day do not provide additional benefit but increase side effects) 1, 4
• Consider adjunctive therapy with:
  • Azathioprine (allows reduction of steroid dose by approximately 45%) 1
  • Tetracycline plus nicotinamide 1
  • Dapsone or sulfonamides 1

Special Considerations

• Bullous pemphigoid is a self-limiting disease that usually remits within 5 years 1
• Mortality rates are significantly higher with systemic corticosteroids compared to topical treatment (HR 1.43) 2
• Systemic corticosteroids also increase the risk of major adverse cardiac events (HR 1.33) and infections (HR 1.33) 2
• Small blisters should be left intact, while larger blisters should be punctured and drained, leaving the blister roof in place 1
• Consider additional measures such as antiseptic baths and appropriate dressings for extensive erosive lesions 1

Monitoring and Follow-up

• Regular follow-up visits should be scheduled: every 2 weeks for the first 3 months, then monthly for the next 3 months, then every 2 months 1
• Monitor for disease activity (blisters, eczematous/urticarial lesions, pruritus) 1
• Consider testing anti-BP180 IgG by ELISA at days 0,60, and 150, as antibody fluctuations may predict outcome 1
• A follow-up visit 3 months after treatment discontinuation is recommended to detect potential relapses 1

Treatment Discontinuation

• Consider discontinuing treatment after 12 months if the patient has been symptom-free for at least 1-6 months on minimal therapy 1
• Be aware that positive direct immunofluorescence or BP180 ELISA >27 U/mL indicates increased risk of relapse 1
• Check for potential adrenal insufficiency after discontinuation, even with topical application 1