When pemphigus vulgaris presents as pemphigus foliaceus (PF) with limited skin involvement, the treatment strategy is tailored to the mild disease extent. This protocol covers patients where the involved body surface area is below 5% and/or the PDAI score is 15 or less.

Clinical scenario: Pemphigus foliaceus with involved BSA below 5% and/or PDAI score ≤ 15 — characterising mild, limited disease. These thresholds directly inform which first-line options are appropriate.

First-Line Management

Several evidence-based first-line strategies exist for mild pemphigus foliaceus. Depending on lesion extent and individual patient factors, options range from topical corticosteroid-based regimens to systemic approaches — with distinct combinations available for each clinical circumstance. The full protocol details which option applies and how to sequence therapy.

Treatment goal: Disease control achieved

References

DOI: 10.1111/jdv.16752

PF with involved body surface area (BSA) <5 %
PDAI score ≤ 15.
Dapsone start with 50 to 100 mg/day, adjusted to clinical response up to 1.5 mg/kg bodyweight.
Dapsone is usually combined with topical corticosteroids (classes III and IV)
Topical corticosteroids (class III, IV) alone if there are only very limited lesions
Systemic corticosteroid therapy with prednisone 0.5–1.0 mg/kg/day
Rituximab (two infusions of 1 g two weeks apart) alone, or associated with topical corticosteroids, or oral corticosteroids (prednisone 0.5 mg/kg/day) with a rapid decrease in order to stop corticosteroids after 3 to 4 months
Disease control achieved

View source ↗

Treatment of Pemphigus Vulgaris in Pemphigus Foliaceus with Involved BSA Below 5%

First-Line Management

References