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与变应性肉芽肿性血管炎相关的威尔斯综合征。

Wells' syndrome associated with churg-strauss syndrome.

作者信息

Lee Soo Hyun, Roh Mi Ryung, Jee Hyunjoong, Chung Kee Yang, Jung Jin Young

机构信息

Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Korea.

出版信息

Ann Dermatol. 2011 Nov;23(4):497-500. doi: 10.5021/ad.2011.23.4.497. Epub 2011 Nov 3.

Abstract

Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features.

摘要

变应性肉芽肿性血管炎(CSS)是一种多系统肉芽肿性血管炎,其特征为外周血嗜酸性粒细胞增多以及嗜酸性粒细胞浸润全身各器官。CSS的皮肤损害主要由可触及的紫癜和结节组成。韦尔斯综合征(WS)是一种罕见的炎症性皮肤病,与复发性肉芽肿性皮炎和嗜酸性粒细胞增多有关。由于这两种疾病都很罕见,它们之间的任何重叠都非常不寻常。在此,我们报告一名CSS患者,该患者最初表现为红斑性荨麻疹斑块、水疱和大疱的皮肤疹。活检时,这些斑块的组织学显示真皮内有嗜酸性粒细胞浸润和“火焰状图形”,这与WS的诊断一致。尽管在我们的患者中观察到的WS与CSS之间的关联可能纯属巧合,但这也可能提示这两种不同疾病有共同的发病机制背景,因为它们有几个共同特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fce/3229945/737e33bc148a/ad-23-497-g001.jpg

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