Dupilumab as Immunomodulatory Rescue for Severe Recalcitrant Pemphigus Vulgaris: A Case Report and Literature Review
Issued Date
2025-01-01
Resource Type
eISSN
11787015
Scopus ID
2-s2.0-105012190465
Journal Title
Clinical Cosmetic and Investigational Dermatology
Volume
18
Start Page
1775
End Page
1782
Rights Holder(s)
SCOPUS
Bibliographic Citation
Clinical Cosmetic and Investigational Dermatology Vol.18 (2025) , 1775-1782
Suggested Citation
Boonpethkaew S., Chanprapaph K. Dupilumab as Immunomodulatory Rescue for Severe Recalcitrant Pemphigus Vulgaris: A Case Report and Literature Review. Clinical Cosmetic and Investigational Dermatology Vol.18 (2025) , 1775-1782. 1782. doi:10.2147/CCID.S535496 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/111545
Title
Dupilumab as Immunomodulatory Rescue for Severe Recalcitrant Pemphigus Vulgaris: A Case Report and Literature Review
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Author's Affiliation
Corresponding Author(s)
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Abstract
Standard treatment of pemphigus vulgaris (PV) includes corticosteroid, immunosuppressants, and biologics such as rituximab, a monoclonal antibody targeting CD20<sup>+</sup> B cells. However, some patients develop resistance to rituximab, requiring alternative therapeutic approaches. We report a 15-year-old female with severe PV who developed rituximab refractoriness after an initially effective response. Despite treatment with a combination of intravenous immunoglobulin, corticosteroid, and immunosup-pressants, the patient failed to achieve disease control. Consequently, dupilumab, an interleukin-4 receptor α antagonist, was initiated on a biweekly regimen. Her lesions showed dramatic improvement, with the pemphigus disease area index (PDAI) reaching 0. Her anti-desmoglein 1 antibody level became negative, and T helper (Th)-2 inflammatory markers, including eosinophil count and immunoglobulin E (IgE) level, was normalized, allowing corticosteroid tapering after the 8th dose (last dose) of dupilumab. She has maintained complete remission for at least 28 weeks with regular follow-ups. We additionally propose possible mechanisms underlying rituximab refractoriness and how dupilumab modulates this treatment response. Our case highlights dupilumab’s potential in modulating Th-2-driven autoantibody production for PV patients with high peripheral eosinophils and IgE levels who have severe disease resistant to corticosteroids or rituximab. Plain Language Summary: This is a report on a case with severe pemphigus vulgaris (PV) unresponsive to rituximab (anti-CD20) and successfully treated with dupilumab (anti-IL4Rα), suggesting a potential role for dupilumab in severe recalcitrant PV.