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Case report: a novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy

Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis that is characterized by autoantibodies against epidermal adhesion proteins causing painful mucosal and skin blistering. Standard treatments for PV include corticosteroids, steroid-sparing immunosuppressants, or intravenous monoclonal...

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Bibliographic Details
Main Authors: Wiedenmayer, Nadine (Author) , Hogrefe, Katharina (Author) , Mihalceanu, Silvia (Author) , Winkler, Julia K. (Author) , Enk, Alexander (Author)
Format: Article (Journal)
Language:English
Published: December 2024
In: The journal of dermatology
Year: 2024, Volume: 51, Issue: 12, Pages: 1665-1668
ISSN:1346-8138
DOI:10.1111/1346-8138.17475
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1111/1346-8138.17475
Verlag, kostenfrei, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/1346-8138.17475
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Author Notes:Nadine Wiedenmayer, Katharina Hogrefe, Silvia Mihalceanu, Julia K. Winkler, Alexander H. Enk (Department of Dermatology, University Hospital Heidelberg)
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Summary:Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis that is characterized by autoantibodies against epidermal adhesion proteins causing painful mucosal and skin blistering. Standard treatments for PV include corticosteroids, steroid-sparing immunosuppressants, or intravenous monoclonal anti-CD20-antibody therapy. The European guidelines suggest high-dose intravenous immunoglobulin (IVIg) therapy as a promising approach for severe or treatment-resistant cases. We report on a 65-year-old woman with severe and recurrent disease who achieved long-term disease stabilization with IVIg treatment. Because of recurrent fatigue and headache, the patient was switched to an alternative IVIg preparation with a novel manufacturing process, thus ensuring high purity and better tolerability. We observed excellent efficacy, yet side effects remained largely unchanged. Further studies are necessary to evaluate the long-term efficacy and tolerability of this new IVIg preparation.
Item Description:Gesehen am 26.02.2025
Physical Description:Online Resource
ISSN:1346-8138
DOI:10.1111/1346-8138.17475

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