Clinical Scorecard: Clinical Case: Utilizing Dupilumab as a Corticosteroid-Sparing Treatment in Severe Mucocutaneous Pemphigus Vulgaris Complicated by Previous Avascular Necrosis and Chronic Kidney Disease

At a Glance

Key Highlights

• Dupilumab was used off-label to minimize corticosteroid exposure in a patient with severe pemphigus vulgaris.
• The patient had a history of avascular necrosis due to high-dose corticosteroid therapy.
• Disease control was achieved with a significant reduction in Pemphigus Disease Area Index scores.
• No adverse events were observed during treatment.
• Dupilumab was combined with a reduced-dose oral prednisone regimen.

Guideline-Based Recommendations

Diagnosis

• Diagnosis confirmed by clinical, immunopathologic, and serologic findings.

Management

• Rituximab is a guideline-supported first-line option for moderate-to-severe pemphigus vulgaris.

Monitoring & Follow-up

• Monitor for disease activity and potential adverse effects of treatments.

Risks

• Consider risks associated with corticosteroid use and potential adverse effects of rituximab.

Patient & Prescribing Data

A 60-year-old woman with severe mucocutaneous pemphigus vulgaris and advanced chronic kidney disease.

Dupilumab was introduced as an adjunctive treatment to minimize corticosteroid use.

Clinical Best Practices

• Prioritize corticosteroid minimization in patients with a history of steroid-related complications.
• Engage in shared decision-making regarding treatment options.

Related Resources & Content

• Clinical Case Report