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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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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author Xiaoyuan Hou
Jia Chen
author_facet Xiaoyuan Hou
Jia Chen
author_sort Xiaoyuan Hou
collection DOAJ
description 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.
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publishDate 2025-01-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Immunology
spelling doaj-art-8226f10f746747b1aaab39f4d9cb63692025-01-13T05:10:43ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-01-011510.3389/fimmu.2024.14811921481192Case report: A rollercoaster journey of pemphigus vegetansXiaoyuan HouJia ChenPemphigus 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.https://www.frontiersin.org/articles/10.3389/fimmu.2024.1481192/fullimmunobullous diseasepemphigus vegetansIL-36 receptor antagonistspesolimabhistopathology
spellingShingle Xiaoyuan Hou
Jia Chen
Case report: A rollercoaster journey of pemphigus vegetans
Frontiers in Immunology
immunobullous disease
pemphigus vegetans
IL-36 receptor antagonist
spesolimab
histopathology
title Case report: A rollercoaster journey of pemphigus vegetans
title_full Case report: A rollercoaster journey of pemphigus vegetans
title_fullStr Case report: A rollercoaster journey of pemphigus vegetans
title_full_unstemmed Case report: A rollercoaster journey of pemphigus vegetans
title_short Case report: A rollercoaster journey of pemphigus vegetans
title_sort case report a rollercoaster journey of pemphigus vegetans
topic immunobullous disease
pemphigus vegetans
IL-36 receptor antagonist
spesolimab
histopathology
url https://www.frontiersin.org/articles/10.3389/fimmu.2024.1481192/full
work_keys_str_mv AT xiaoyuanhou casereportarollercoasterjourneyofpemphigusvegetans
AT jiachen casereportarollercoasterjourneyofpemphigusvegetans