Clinical Report: Utilizing Dupilumab as a Corticosteroid-Sparing Treatment in Severe Mucocutaneous Pemphigus Vulgaris

Background

Pemphigus vulgaris is a rare autoimmune blistering disease that poses significant treatment challenges, particularly in patients with prior corticosteroid toxicity. Standard treatment typically involves systemic corticosteroids and steroid-sparing agents, but complications such as avascular necrosis can complicate management.

Data Highlights

No numerical data or trial data available in the article.

Key Findings

• A 60-year-old woman with severe pemphigus vulgaris had a history of avascular necrosis due to corticosteroid use.
• Dupilumab was used off-label as an adjunct to oral prednisone for corticosteroid minimization.
• The Pemphigus Disease Area Index score decreased from 111 to 22 within six weeks of treatment, as reported in the case.
• Complete re-epithelialization was achieved by approximately month 4 with a reduced prednisone dose.
• No adverse events were reported during the treatment period.

Clinical Implications

The case illustrates the potential of dupilumab as a corticosteroid-sparing option in patients with pemphigus vulgaris who have experienced steroid-related complications. Further research is needed to establish guidelines for patient selection and long-term management.

Conclusion

This case supports the feasibility of using dupilumab in managing pemphigus vulgaris while minimizing corticosteroid exposure, although more evidence is required to guide its use.

Related Resources & Content

1. Didona et al., Journal of the European Academy of Dermatology and Venereology, 2020 -- Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus
2. Efficacy, safety, and B-cell depletion capacity of 3 rituximab dosing regimens in the treatment of moderate-to-severe pemphigus vulgaris and pemphigus foliaceus: A 52-week clinical trial, ScienceDirect, 2025
3. Concurrent Oral Pemphigus Vulgaris and Herpes Simplex Virus Infection in a Young Patient with Crohn’s Disease: A Unique Case of Oral Lesions During Anti-TNF Alpha and Immunomodulatory Treatment
4. Clinical Rheumatology — Management and Long-Term Outcomes in Children with Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS): A Review of Case Studies
5. Frontiers in Medicine — Editorial: Exploring Cutaneous Drug-Related and Drug-Associated Adverse events: from Clinical Insights to Therapeutic Management
6. The ASCO — Diagnosing and Treating Chronic Mucocutaneous Graft-vs-Host Disease
7. Concurrent Oral Pemphigus Vulgaris and Herpes Simplex Virus Infection in a Young Patient with Crohn’s Disease
8. Clinical Rheumatology — Management and Long-Term Outcomes in Children with Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS): A Review of Case Studies
9. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV) - Joly - 2020 - Journal of the European Academy of Dermatology and Venereology - Wiley Online Library
10. Efficacy, safety, and B-cell depletion capacity of 3 rituximab dosing regimens in the treatment of moderate-to-severe pemphigus vulgaris and pemphigus foliaceus: A 52-week clinical trial - ScienceDirect
11. DUPIXENT (dupilumab) injection, for subcutaneous use — Highlights of Prescribing Information