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CASE REPORT article

Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 15 - 2024 | doi: 10.3389/fimmu.2024.1481192
This article is part of the Research Topic Autoimmunity: novel insights and future perspectives View all 32 articles

Article type: Case report A Rollercoaster Journey of Pemphigus Vegetans

Provisionally accepted
Xiaoyuan Hou Xiaoyuan Hou Jia Chen Jia Chen *
  • Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China

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

    Pemphigus vegetans (P Veg), the rarest subtype of pemphigus, is characterized by vegetative plaques, primarily affecting intertriginous areas. The most common autoantibodies target desmoglein 3 (Dsg3). A 60-year-old female patient presented with well-demarcated red vegetative plaques on her feet, vulva, and thigh, accompanied by surrounding pustules. Histopathological examination revealed epidermal hyperplasia with significant infiltration of neutrophils and eosinophils in the dermis. Enzyme-linked immunosorbent assay (ELISA) showed elevated anti-Dsg3 antibodies (203.2 U/mL), and immunohistochemical staining confirmed positive expression of anti-Dsg3 IgG antibodies in keratinocytes. The patient was diagnosed with P Veg and achieved remission after treatment with either 900 mg of intravenous spesolimab or oral methylprednisolone.

    Keywords: immunobullous disease, Pemphigus vegetans, IL-36 receptor antagonist, spesolimab, histopathology

    Received: 15 Aug 2024; Accepted: 23 Dec 2024.

    Copyright: © 2024 Hou and Chen. 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: Jia Chen, Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China

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