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大疱性类天疱疮与多系统萎缩：病例系列

Bullous Pemphigoid Associated with Multiple System Atrophy: Case Series.

作者信息

Snedden Andrew, Sharif Jennifer, Newsham John, Kobylecki Christopher

机构信息

Department of Neurology Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust Salford United Kingdom.

Centre for Musculoskeletal Research University of Manchester Manchester United Kingdom.

出版信息

Mov Disord Clin Pract. 2021 Feb 25;8(3):445-448. doi: 10.1002/mdc3.13160. eCollection 2021 Apr.

DOI:10.1002/mdc3.13160
PMID:33816675
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8015901/
Abstract

BACKGROUND

Bullous pemphigoid (BP) is an autoimmune blistering dermatosis associated with a number of neurological conditions, including idiopathic Parkinson's disease (IPD). Only 1 case of BP in a patient with multiple system atrophy (MSA) has been reported.

CASES

We report 3 cases of men with probable MSA who developed bullous pemphigoid at a latency of 4-6 years from MSA symptom onset.

CONCLUSIONS

Skin α-synuclein deposition in neurodegenerative conditions such as IPD and MSA may be a potential substrate for the exposure of BP-related antigens. Alternatively, central neurodegeneration may expose antigens as a substrate for cross-reactivity and BP pathogenesis. Our report suggests an association between BP and MSA, in addition to the previously documented association with IPD.

摘要

背景

大疱性类天疱疮（BP）是一种自身免疫性水疱性皮肤病，与多种神经系统疾病相关，包括特发性帕金森病（IPD）。仅报告过1例多系统萎缩（MSA）患者发生BP的病例。

病例

我们报告3例可能患有MSA的男性患者，他们在MSA症状出现后4 - 6年出现了大疱性类天疱疮。

结论

在IPD和MSA等神经退行性疾病中，皮肤α-突触核蛋白沉积可能是暴露BP相关抗原的潜在底物。或者，中枢神经退行性变可能暴露抗原作为交叉反应和BP发病机制的底物。我们的报告表明，除了先前记录的与IPD的关联外，BP与MSA之间也存在关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/62d1/8015901/bebfae4257a1/MDC3-8-445-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/62d1/8015901/3da4e76693a2/MDC3-8-445-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/62d1/8015901/bebfae4257a1/MDC3-8-445-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/62d1/8015901/3da4e76693a2/MDC3-8-445-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/62d1/8015901/bebfae4257a1/MDC3-8-445-g001.jpg

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Dermatol Ther. 2020 Jul;33(4):e13790. doi: 10.1111/dth.13790. Epub 2020 Jul 14.
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A Systematic Review of Drug-Induced Pemphigoid.药物性类天疱疮的系统评价
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Demographics and Autoantibody Profiles of Pemphigoid Patients with Underlying Neurologic Diseases.
迟发性家族性共济失调中的脊髓小脑共济失调45型：来自印度次大陆的首例报告及一种新变异型
Mov Disord Clin Pract. 2022 Oct 10;9(8):1140-1143. doi: 10.1002/mdc3.13580. eCollection 2022 Nov.
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Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study.在体内研究中发现，不同的突触核蛋白病临床变异型α-突触核蛋白沉积物存在差异。
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Mortality and Comorbidity Profiles of Patients with Bullous Pemphigoid in Korea.韩国大疱性类天疱疮患者的死亡率和合并症概况。
Ann Dermatol. 2018 Feb;30(1):13-19. doi: 10.5021/ad.2018.30.1.13. Epub 2017 Dec 26.
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A multi-hit hypothesis of bullous pemphigoid and associated neurological disease: Is HLA-DQB1*03:01, a potential link between immune privileged antigen exposure and epitope spreading?大疱性类天疱疮和相关神经疾病的多打击假说：HLA-DQB1*03:01 是否是免疫特权抗原暴露与表位扩展之间的潜在联系？
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