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REVIEW article

Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1481093
This article is part of the Research Topic Molecular Characterisation of Autoimmune Diseases View all articles

Autoimmune pemphigus: difficulties in diagnosis and the molecular mechanisms underlying the disease

Provisionally accepted
Olga Simionescu Olga Simionescu 1*Sorin Ioan TRudorache Sorin Ioan TRudorache 2
  • 1 Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
  • 2 Faculty of Medicine, Titu Maiorescu University, Bucharest, Bucharest, Romania

The final, formatted version of the article will be published soon.

    Recently recognised as a desmosomal disorder, autoimmune pemphigus remains severe in some of its forms, such as pemphigus vulgaris. This review is divided into four parts. "Cellular and molecular mechanisms in autoimmune pemphigus" discusses in detail antigenic targets, antibodies, immunological and genetic mechanisms of apoptosis and the involvement of cells and organelles (keratinocytes, lymphocytes, eosinophils and neutrophils) in different forms of pemphigus. These advances have led to today's first-line biologic therapy for pemphigus. The section "Specific features in the diagnosis of immune pemphigus" deals with the clinical diagnostic clues (enanthema, intertrigo, pruritus, distribution of lesions). The third section, "Characteristics and challenges in different types of pemphigus", focuses on the importance of using standardised diagnostic criteria in paraneoplastic pemphigus and pemphigus herpetiformis, the specific and difficult situations of differentiation between bullous lupus and autoimmune Senear-Usher pemphigus, between IgA forms of pemphigus or differentiation with other autoimmune diseases or neutrophilic dermatoses. The possibility of subtype cross-reactivity in pemphigus is also discussed, as is the diagnosis and course of the disease in pregnant women. The final section is an update of the "gold standard for the diagnosis and evaluation of autoimmune pemphigus", the role and place of direct immunofluorescence and additional serological tests. This revision is the first to combine the difficulties in clinical diagnosis with new molecular insights. It provides a comprehensive overview of recent advances in the understanding of autoimmune pemphigus, bridging the clinical challenges and complexities of diagnosing different forms of pemphigus, and is a valuable resource for clinicians caring for patients with pemphigus.

    Keywords: Pemphigus, Direct immunofluorescence, Bullous diseases, Desmogleins, diagnostic

    Received: 15 Aug 2024; Accepted: 02 Dec 2024.

    Copyright: © 2024 Simionescu and TRudorache. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Olga Simionescu, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.