Pemphigus vulgaris
ICD-10 L10.0 · ICD-11 EB40.0

Treatment of Pemphigus Vulgaris with Involved BSA Below 5% and Limited Oral Lesions

This protocol covers first-line management of pemphigus vulgaris presenting as mild disease — with limited body surface area involvement and oral lesions that do not impair eating or require analgesics.

Clinical Scenario

Pemphigus vulgaris with involved body surface area (BSA) below 5% and limited oral lesions not impairing food intake or requiring analgesics, and/or a PDAI score of 15 or less.

Treatment Approach

First-line options for this mild presentation include targeted biologic therapy — used alone or combined with a short corticosteroid course — as well as systemic corticosteroid-based regimens with or without adjuvant immunosuppression.

Full agent selection, sequencing, and dosing details are available in the complete structured protocol below.

Treatment Goal

Disease control achieved.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1111/jdv.16752

PV with involved body surface area (BSA) < 5% and limited oral lesions not impairing food intake or requiring analgesics.

PDAI score ≤ 15.

Rituximab: two infusions of 1g two weeks apart, alone, or associated with oral prednisone 0.5 mg/kg/day with a rapid decrease in order to stop corticosteroids after 3 or 4 months.

Systemic corticosteroids: prednisone 0.5–1.0 mg/kg/day with or without azathioprine (2.0 mg/kg/d), or mycophenolate mofetil 2 g/day or mycophenolate sodium 1440 mg/d.

Disease control achieved.

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