Bullous pemphigoid
ICD-10 L12.0 · ICD-11 EB41.0

Treatment of Bullous Pemphigoid with 10 or Fewer New Blisters per Day and BPDAI Below 57 (Mild–Moderate Disease)

This protocol addresses bullous pemphigoid presenting with limited blistering activity — specifically, no more than 10 new blisters per day alongside a Bullous Pemphigoid Disease Area Index (BPDAI) score below 57, covering both mild and moderate severity strata.

Mild disease is defined by a BPDAI score below 20. Moderate disease falls between a BPDAI of 20 and below 57. Both subgroups share the hallmark of 10 or fewer new blisters daily, and may also include limited non-bullous inflammatory lesions across different body areas. Accurate BPDAI scoring guides the placement of the patient within this spectrum and informs treatment intensity.

The primary target is disease control — the point at which new lesions and pruritic symptoms cease to form and established lesions begin to heal. Evidence indicates that a substantial proportion of patients in both the mild and moderate groups can reach this endpoint within three weeks of initiating first-line therapy.

First-line management centres on corticosteroid therapy, which may be delivered topically at high potency or orally at medium dose — the two principal pathways recommended for this severity range.

Additional options and their combinations 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).
  • High-potency topical CS with an initial dose of clobetasol propionate of 20 to 30 g per day, applied twice or once a day (4.68 ± 0.62).
  • Medium doses of oral CS, that is, oral prednisone 0.5 mg/kg/day (4.90 ± 0.36).
  • Three other therapeutic options may be considered either alone or in combination with topical or oral CS.
  • Doxycycline 200 mg daily (3.83 ± 1.37).
  • Methotrexate, 10–12.5 mg/week initially (3.54 ± 1.55) (if no contraindication including renal insufficiency, otherwise reduce doses or avoid).
  • Dapsone (1 mg to 1.5 mg/kg daily) (3.83 ± 1.30).
  • 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.
  • A recent prospective observational multicentre study has indicated that a 0.5 mg/kg/day starting dose of prednisone allows disease control to be achieved at day 21 in 75% and 69% of patients with mild and moderate BP, respectively, but in only 46% of patients with severe BP.