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Case report: A rollercoaster journey of pemphigus vegetans
1Department of Rheumatology, Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China.
None:
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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