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
Published in:Journal of dermatology 2024-12, Vol.51 (12), p.1665-1668
Main Authors: Wiedenmayer, Nadine, Hogrefe, Katharina, Mihalceanu, Silvia, Winkler, Julia K., Enk, Alexander H.
Format: Article
Language:English
Subjects:
adverse effects
Aged
Autoantibodies
autoimmune bullous dermatoses
CD20 antigen
Concise Communication
Corticosteroids
Drug Substitution
Female
Humans
Immunoglobulins
Immunoglobulins, Intravenous - administration & dosage
Immunoglobulins, Intravenous - therapeutic use
Immunologic Factors - administration & dosage
Immunologic Factors - therapeutic use
Immunosuppressive agents
Immunotherapy
Intravenous administration
intravenous immunoglobulins
IVIg
Pemphigus
Pemphigus - drug therapy
Pemphigus - immunology
pemphigus vulgaris
Recurrence
Severity of Illness Index
Treatment Failure
Treatment Outcome
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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.
ISSN:0385-2407
1346-8138
1346-8138
DOI:10.1111/1346-8138.17475