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Giornale Italiano di Dermatologia e Venereologia 2020 Oct 05

DOI: 10.23736/S0392-0488.20.06694-8

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

B-cell targeted therapies in pemphigus

Roberto MAGLIE 1 , Emiliano ANTIGA 1, Aimee S. PAYNE 2

Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy; 2 Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA


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Pemphigus is a rare autoimmune disease of the skin, characterized by autoantibodies targeting adhesion proteins of the epidermis, in particular desmoglein 3 and desmoglein 1, that cause the loss of cell-cell adhesion and the formation of intraepidermal blisters. Given that these autoantibodies are both necessary and sufficient for pemphigus to occur, the goal of pemphigus therapy is the elimination of autoreactive Bcells responsible for autoantibody production. Rituximab, an anti-CD20 monoclonal antibody, was the first targeted B-cell therapy approved for use in pemphigus, and is now considered the frontline therapy for new onset disease. One limitation of this treatment is that it targets both autoreactive and non - autoreactive B-cells, which accounts for the increased risk of serious infections in treated patients. In addition, most rituximab-treated patients experience disease relapse, highlighting the need of new therapeutic options. This review provides a concise overview of rituximab use in pemphigus and discusses new B-cell and antibody-directed therapies undergoing investigation in clinical studies.


KEY WORDS: Pemphigus; Rituximab; B-cells, B-cell targeted therapies

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