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大疱性类天疱疮的临床表现多样性。

Clinical manifestation variability of bullous pemphigoid.

出版信息

Bratisl Lek Listy. 2024;125(11):685-692. doi: 10.4149/BLL_2024_104.

Abstract

BACKGROUND

Bullous pemphigoid (BP) is a rare autoimmune blistering disease predominantly affecting the elderly population.

OBJECTIVES

The present study aims to identify clinical factors that may influence outcomes of BP, including skin phenotype, serology, mucosal involvement, pruritus, and triggers.

METHODS

A retrospective analysis was conducted on 70 cases with BP registered from January 2019 to December 2022. The Bullous Pemphigoid Disease Activity Index (BPDAI) score was used to assess disease intensity. The BPDAI-Pruritus score and a modified Brest questionnaire were used to document pruritus. Anti-BP180 and anti-BP230 autoantibodies were regularly recorded. Peripheral blood eosinophil counts were documented during flare-up and remission phases of BP.

RESULTS

Of the cases, 81.4% were identified with bullous BP, 12.9% with nonbullous BP and 5.7% with localized BP. Oral involvement was documented in 17.1% of cases. Increased peripheral eosinophilia was prominent in the nonbullous phenotype and returned to normal level during remission in both phenotypes.

CONCLUSION

The outcomes of BP depended on the disease phenotype and trigger types. Bullous BP showed more intense disease activity, while nonbullous BP demonstrated more intense pruritus. BP associated with diabetes mellitus (DM) or psoriasis manifested as a more severe disease, predominantly with the bullous phenotype and pruritus, compared to cases without comorbidities. New triggers, including SARS-CoV-2 infection and vaccination, were documented (Tab. 6, Ref. 43). Text in PDF www.elis.sk Keywords: bullous pemphigoid, nonbullous pemphigoid, pruritus, comorbidity, eosinophilia, new triggers.

摘要

背景

大疱性类天疱疮(BP)是一种罕见的自身免疫性水疱病,主要影响老年人群。

目的

本研究旨在确定可能影响 BP 结局的临床因素,包括皮肤表型、血清学、黏膜受累、瘙痒和诱因。

方法

对 2019 年 1 月至 2022 年 12 月登记的 70 例 BP 患者进行回顾性分析。采用大疱性类天疱疮疾病活动指数(BPDAI)评分评估疾病严重程度。采用 BPDAI-瘙痒评分和改良 Brest 问卷评估瘙痒。定期记录抗 BP180 和抗 BP230 自身抗体。记录 BP 发作和缓解期外周血嗜酸性粒细胞计数。

结果

在这些病例中,81.4%为大疱性 BP,12.9%为非大疱性 BP,5.7%为局限性 BP。17.1%的病例有口腔受累。非大疱性表型中外周血嗜酸性粒细胞增多更为明显,在两种表型的缓解期均恢复正常水平。

结论

BP 的结局取决于疾病表型和诱因类型。大疱性 BP 表现出更严重的疾病活动,而非大疱性 BP 表现出更严重的瘙痒。与无合并症的病例相比,BP 合并糖尿病(DM)或银屑病表现为更严重的疾病,主要表现为大疱性表型和瘙痒。还记录了新的诱因,包括 SARS-CoV-2 感染和疫苗接种(表 6,参考文献 43)。

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