Next-line protocol

Mild to Moderate Bullous Pemphigoid: What to Do When Initial Treatment Fails to Achieve Disease Control

This protocol applies to patients with bullous pemphigoid presenting with 10 or fewer new blisters per day and a BPDAI score below 57 — mild disease (BPDAI < 20) or moderate disease (BPDAI ≥ 20 and < 57) — whose first-line therapy has not achieved disease control.

Clinical Scenario

Mild to moderate bullous pemphigoid is characterised by the occurrence of 10 or fewer new blisters per day, or the presence of few non-bullous inflammatory lesions in different localisations. Severity is quantified using the BPDAI scoring system: a score below 20 defines mild disease, while a score from 20 to below 57 defines moderate disease.

Previous Treatment & Failure Condition

First-line therapy for mild to moderate bullous pemphigoid includes high-potency topical corticosteroids (clobetasol propionate) or medium-dose oral prednisone, with additional agents such as doxycycline, methotrexate, or dapsone considered alone or in combination.

This protocol is indicated when the above first-line regimen has not achieved disease control — that is, when new lesions continue to form, pruritic symptoms persist, and established lesions have not begun to heal within the expected timeframe.

Next-Step Approach & Treatment Goal

Clinical goal: Disease control — defined as the point at which new lesions and pruritic symptoms cease to form and established lesions begin to heal.

The approach centres on optimising corticosteroid therapy based on what the patient is currently receiving — whether topical, oral, or through a combined strategy. The specific adjustments are detailed in the full protocol.

References

It is recommended to define mild/moderate BP as the occurrence of 10 or less new blisters per day or by the presence of few non-bullous inflammatory lesions in different localizations.

It is recommended to use the BPDAI scoring system; mild BP corresponds to a severity score lower than 20 points, and moderate BP corresponds to a BPDAI score < 57 points (4.76 ± 0.59).

An increasing dose of topical CS (up to 40 g/day) is recommended in patients receiving less than 40 g/day of clobetasol propionate 0.05% who do not achieve disease control within 1–3 weeks.

In patients who do not achieve disease control within 1–3 weeks with 0.5 mg/kg prednisone, it may be recommended to either increase the dose of prednisone up to 0.75 mg/kg/day (4.62 ± 0.87) or to add topical CS in addition to the 0.5 mg/kg/day dose of prednisone (4.75 ± 0.60) based on recent evidence.

According to the consensus statement, CDA is defined as the point at which new lesions or pruritic symptoms cease to form and established lesions begin to heal.

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