This protocol addresses severe bullous pemphigoid, defined by 10 or more new blisters forming daily across multiple anatomical sites, and/or a BPDAI score of 57 or higher. At this degree of severity, initial first-line therapy may not bring disease activity under control within the expected window.
First-line options for severe bullous pemphigoid include high-potency topical corticosteroid clobetasol propionate applied across the body, or oral prednisone at a medium-to-high starting dose. With the oral prednisone approach at its initial dose, disease control is reached at day 21 in approximately 46% of severe patients — meaning the majority do not meet the disease control target on this regimen alone. This protocol defines the structured next step for those patients.
When the initial oral prednisone dose does not achieve control, the regimen involves either a secondary adjustment to the oral corticosteroid or the addition of topical corticosteroid therapy alongside the systemic agent. The full decision algorithm, complete options, and sequencing are available via the protocol.
Control of disease activity: new lesions and pruritic symptoms cease to form, and established lesions begin to heal.
It is recommended to define severe BP by the occurrence of equal or more than 10 new blisters daily on multiple anatomical sites and/or a BPDAI score ≥ 57 (4.80 ± 0.50).
Secondarily increase in oral prednisone to 0.75 mg/kg/day, or add topical CS in addition to the 0.5 mg/kg/day dose of prednisone.
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