Moderate to Severe Pemphigus When First-Line Therapy Has Not Achieved Disease Control

This protocol covers the escalation step for patients with pemphigus vulgaris or foliaceus with significant skin involvement who have not reached disease control after 3 to 4 weeks on first-line treatment.

Clinical Scenario

Pemphigus (vulgaris or foliaceus) with skin lesions involving more than 5% of body surface area. Moderate disease is defined by a PDAI score above 15 and up to 45; severe disease by a PDAI score above 45.

First-Line Treatment — Target Not Reached

The preceding treatment line — which included first-line options such as rituximab with systemic corticosteroids, or systemic corticosteroids alone or combined with an immunosuppressant — did not achieve the primary goal: disease control by week 3–4. This protocol defines the next clinical step after that failure.

Escalation Approach (Partial Overview)

Second-line escalation for moderate and severe pemphigus involves adjusting the corticosteroid regimen and/or adding targeted immunotherapy, with the specific pathway guided by what the initial first-line regimen consisted of. The complete step-by-step algorithm is in the full protocol.

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.
Initially treated with rituximab and prednisone → increase the prednisone dose up to 1.5 mg/kg/d or → intravenous corticosteroids pulses
Initially treated with systemic corticosteroids (1.0 mg/kg/d) alone → increase the prednisone dose to 1.5 mg/kg/d PLUS rituximab (2x1g) or → add immunosuppressant (azathioprine 1 to 2.5 mg/kg/d or mycophenolate mofetil 2g/d or mycophenolate sodium 1440 mg/d)
Initially treated with prednisone (1.5 mg/kg/d) alone → add rituximab (2x1g) or → add immunosuppressant (azathioprine 1 to 2.5 mg/kg/d or mycophenolate mofetil 2g/d or mycophenolate sodium 1440 mg/d)
Disease control

View source ↗