当前位置: X-MOL 学术JAMA Dermatol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Overlap of Bullous, Anti-Laminin-332, and Anti-p200 Pemphigoid With Concomitant Anti-Contactin-1-Positive Inflammatory Polyneuropathy Treated With Intravenous Immunoglobulins as a Manifestation of Epitope Spreading.
JAMA Dermatology ( IF 10.9 ) Pub Date : 2019-05-01 , DOI: 10.1001/jamadermatol.2018.5536
Alexander Wald 1 , Enno Schmidt 2 , Ferdinand Toberer 1 , Alexander Gutschalk 3 , Kristin Rentzsch 4 , Alexander H Enk 1 , Jochen H O Hoffmann 1
Affiliation  

Bullous pemphigoid (BP), anti-p200 pemphigoid, and anti–laminin-332 mucous membrane pemphigoid are distinct subepidermal autoimmune bullous diseases (AIBDs).1 Anti–contactin-1 (anti-CNTN1)–positive inflammatory polyneuropathy (IP) is a recently described entity with clinical similarities to chronic inflammatory demyelinating polyneuropathy, which manifests pathologically as a disruption of the paranodal architecture.2,3 The patient in this case initially presented with BP, and after having been successfully treated and living symptom-free for 5 years, he developed an overlap of BP, anti-p200 pemphigoid, and anti–laminin-332 mucous membrane pemphigoid with an anti-CNTN1–positive IP in close temporal proximity. We hypothesize an underlying disease-modifying intermolecular epitope-spreading (ES) phenomenon.



中文翻译:

大疱,抗层粘连蛋白332和抗p200天疱疮与伴随的抗Contactin-1阳性炎性多发性神经炎的重叠治疗,静脉免疫球蛋白治疗可作为抗原决定簇的表现。

大疱性类天疱疮(BP),抗p200类天疱疮和抗laminin-332粘膜类天疱疮是不同的表皮下自身免疫性大疱性疾病(AIBD)。1抗接触素1(anti-CNTN1)阳性炎症性多发性神经病(IP)是最近描述的与慢性炎症性脱髓鞘性多发性神经病具有临床相似性的实体,其病理表现为淋巴结旁结构的破坏。2 ,3-在这种情况下,该患者最初患有BP,在成功治疗并且没有出现任何症状5年后,他出现了BP,抗p200类天疱疮和抗层粘连蛋白332粘膜类天疱疮以及一种抗高血压药物的重叠现象。 CNTN1阳性IP在时间上非常接近。我们假设潜在的疾病修饰分子间表位扩展(ES)现象。

更新日期:2019-05-19
down
wechat
bug