Objective:

To report a case of severe mucocutaneous pemphigus vulgaris treated with dupilumab as a corticosteroid-sparing approach in a patient with prior steroid toxicity and treatment-related constraints.

Approach:

• Treatment Decision: Rituximab was discussed as a guideline-supported first-line option; however, the patient declined it after counseling about its expected benefits and potential adverse effects. Therefore, dupilumab was introduced off-label as an adjunctive corticosteroid-sparing approach alongside oral prednisone.

Key Findings:

• Dupilumab assisted in minimizing corticosteroid use while achieving disease control.
• Complete re-epithelialization occurred by approximately month 4 with a reduced prednisone dose of 7.5 mg/day.
• No adverse events were reported during the treatment period.

Interpretation:

This case illustrates the potential for dupilumab to assist in corticosteroid minimization in pemphigus vulgaris patients facing treatment-related constraints.

Limitations:

• Further evidence is needed regarding the selection criteria for patients receiving dupilumab therapy.
• The long-term risk of relapse and the optimal duration of therapy remain to be established.

Conclusion:

Dupilumab may represent a potential option for corticosteroid-sparing treatment in selected patients with pemphigus vulgaris.