Case report: A rollercoaster journey of pemphigus vegetans

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, vu...

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Bibliographic Details
Main Authors: Xiaoyuan Hou, Jia Chen
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-01-01
Series:Frontiers in Immunology
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Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2024.1481192/full
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Summary: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 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.
ISSN:1664-3224