Pemphigus: currently important components of pathogenesis and therapy


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Abstract

Pemphigus is a group of bullous dermatoses, characterized by acantholysis of epidermal cells, with circulating autoantibodies against desmosome antigens in the stratified squamous epithelium. The article describes the main mechanisms of the development of the disease: genetic factors, the influence of taking certain drugs, the role of autoantigens and autoantibodies, the mechanisms of cell signaling, the influence of cellular immunity. Current treatment options for this pathology are also presented; they include immunosuppressants such as glucocorticosteroids and corticosteroid-sparing agents - azathioprine, mycophenolate mofetil, cyclophosphamide. New therapies are aimed at reducing circulating autoantibodies against desmogleins; one of them is rituximab, a chimeric anti-CD20 type I monoclonal antibody, which can bind to the CD20 antigen and reduce the level of activated B-lymphocytes.

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About the authors

N. V Zilberberg

Ural Research Institute of Dermatovenereology and Immunopathology

Yekaterinburg, Russia

M. M Kokhan

Ural Research Institute of Dermatovenereology and Immunopathology

Yekaterinburg, Russia

Anastasia N. Shakirova

Ural Research Institute of Dermatovenereology and Immunopathology

Email: shakirova.a96@yandex.ru
Junior Researcher, Scientific Clinical Department of Dermatology Yekaterinburg, Russia

M. A Shpak

Ural Research Institute of Dermatovenereology and Immunopathology

Yekaterinburg, Russia

I. D Kuznetsov

Academician E.A. Vagner Perm State Medical University

Perm, Russia

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