{"title":"大疱性类天疱疮的临床表现变异性。","authors":"Danka Svecova, Jana Nemsovska, Pavel Babal","doi":"10.4149/BLL_2024_104","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Bullous pemphigoid (BP) is a rare autoimmune blistering disease predominantly affecting the elderly population.</p><p><strong>Objectives: </strong>The present study aims to identify clinical factors that may influence outcomes of BP, including skin phenotype, serology, mucosal involvement, pruritus, and triggers.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":55328,"journal":{"name":"Bratislava Medical Journal-Bratislavske Lekarske Listy","volume":"125 11","pages":"685-692"},"PeriodicalIF":1.5000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical manifestation variability of bullous pemphigoid.\",\"authors\":\"Danka Svecova, Jana Nemsovska, Pavel Babal\",\"doi\":\"10.4149/BLL_2024_104\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Bullous pemphigoid (BP) is a rare autoimmune blistering disease predominantly affecting the elderly population.</p><p><strong>Objectives: </strong>The present study aims to identify clinical factors that may influence outcomes of BP, including skin phenotype, serology, mucosal involvement, pruritus, and triggers.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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). 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引用次数: 0
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:本研究旨在确定可能影响大疱性类天疱疮预后的临床因素,包括皮肤表型、血清学、粘膜受累、瘙痒和诱发因素: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)评分来评估疾病强度。BPDAI-瘙痒评分和改良的布雷斯特问卷用于记录瘙痒情况。定期记录抗 BP180 和抗 BP230 自身抗体。在 BP 爆发期和缓解期记录外周血嗜酸性粒细胞计数:结果:81.4%的病例被确诊为牛皮癣,12.9%为非牛皮癣,5.7%为局部牛皮癣。17.1%的病例有口腔受累记录。外周嗜酸性粒细胞增多在非大疱性表型中很突出,在两种表型的缓解期均恢复到正常水平:结论:BP 的预后取决于疾病表型和诱因类型。结论:BP 的结果取决于疾病表型和诱因类型。大疱性 BP 表现出更强烈的疾病活动,而非大疱性 BP 则表现出更强烈的瘙痒。与无并发症的病例相比,伴有糖尿病(DM)或银屑病的 BP 表现为更严重的疾病,主要表现为牛皮癣表型和瘙痒。新的诱发因素包括 SARS-CoV-2 感染和疫苗接种(参考文献 43,表 6)。PDF 格式的文本 www.elis.sk 关键词: 大疱性类天疱疮、非大疱性类天疱疮、瘙痒、合并症、嗜酸性粒细胞增多、新的诱发因素。
Clinical manifestation variability of bullous pemphigoid.
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.
期刊介绍:
The international biomedical journal - Bratislava Medical Journal
– Bratislavske lekarske listy (Bratisl Lek Listy/Bratisl Med J) publishes
peer-reviewed articles on all aspects of biomedical sciences, including
experimental investigations with clear clinical relevance, original clinical
studies and review articles.