First-Line Treatment of Pemphigus Vulgaris or Foliaceus with Skin Lesions Involving More Than 5% of Body Surface Area

Pemphigus vulgaris and pemphigus foliaceus with substantial cutaneous involvement — defined by skin lesions covering more than 5% of body surface area, or by a Pemphigus Disease Area Index (PDAI) score in the moderate or severe range — require a structured first-line approach aimed at achieving disease control within weeks.

Clinical Scenario

This protocol applies to pemphigus (vulgaris or foliaceus) when skin lesions involve more than 5% of body surface area. Disease burden is classified further by PDAI score: moderate pemphigus corresponds to a PDAI above 15 and up to 45; severe pemphigus corresponds to a PDAI above 45.

Treatment Approach

First-line management for moderate and severe pemphigus involves biologic and/or immunosuppressive systemic therapy — with the specific agents, combination strategy, and tapering plan detailed in the full protocol.

Treatment goal: disease control at weeks 3–4

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/jdv.16752

And/or skin lesions >5% BSA.
Moderate pemphigus PDAI score > 15 and ≤ 45.
Severe pemphigus: PDAI score > 45.
Rituximab: two infusions of 1g two weeks apart, associated with systemic corticosteroids (prednisone 1 mg/kg/day) with a progressive tapering in order to stop corticosteroids after 6 months.
Systemic corticosteroids: oral prednisone 1 to 1.5 mg/kg/day alone or associated with an immunosuppressant (azathioprine 1 to 2.5 mg/kg/d or mycophenolate mofetil 2g/d or mycophenolate sodium 1440 mg/d).
Disease control at week 3–4.

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