The Transition from Pemphigus Foliaceus to Pemphigus Vegetans—An Intriguing Phenomenon within the Spectrum of Autoimmune Blistering Diseases: A Case Report

Dermato Pub Date : 2024-06-14 DOI:10.3390/dermato4020007
O. Orzan, L. Popa, Iulia Badiu, Ana Ion, C. Giurcaneanu, Beatrice Bălăceanu-Gurău, Irina Tudose
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Abstract

Pemphigus vegetans and pemphigus foliaceus are rare autoimmune blistering diseases characterized by the disruption of desmosomal adhesion proteins, particularly desmoglein 3 and desmoglein 1. We report the case of a 62-year-old male who presented initially with scaly red plaques posing several diagnostic challenges. A histopathological examination revealed subcorneal acantholysis, matching the suspected clinical diagnosis of pemphigus foliaceus. The patient progressed, developing vegetating plaques, and a new biopsy was performed. The new histopathological and direct immunofluorescence exams were consistent with pemphigus vegetans. This case highlights the diagnostic challenges posed by the transition of pemphigus foliaceus to its vegetating form. We discuss the role of desmogleins in the pathogenesis of pemphigus and explore potential therapeutic strategies targeting these specific autoantigens.
从叶状天疱疮到植物性天疱疮的转变--自身免疫性水疱病谱中的一个有趣现象:病例报告
植物性丘疹痤疮和叶面丘疹痤疮是罕见的自身免疫性大疱性疾病,其特征是去疱疹体粘附蛋白(尤其是去疱疹素 3 和去疱疹素 1)的破坏。 我们报告了一例 62 岁男性患者的病例,他最初出现鳞屑状红色斑块,这给诊断带来了一些挑战。组织病理学检查显示,患者角质层下出现棘层溶解,这与叶面丘疹性荨麻疹的临床诊断相吻合。患者病情发展,出现了植被性斑块,于是进行了新的活组织检查。新的组织病理学和直接免疫荧光检查结果与植物性天疱疮一致。本病例凸显了由叶状天疱疮转变为植物型天疱疮所带来的诊断挑战。我们讨论了去酪氨酸蛋白在丘疹性荨麻疹发病机制中的作用,并探讨了针对这些特异性自身抗原的潜在治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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