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变应性肉芽肿性血管炎

Churg-strauss syndrome.

作者信息

Ghosh Subhasish, Bhattacharya Maitreyee, Dhar Sandipan

机构信息

Consultant Pulmonologist, AMRI Hospitals, Dhakuria, Kolkata, India .

出版信息

Indian J Dermatol. 2011 Nov;56(6):718-21. doi: 10.4103/0019-5154.91836.

Abstract

Churg-Strauss syndrome (CSS) is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%). We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010). The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS).

摘要

变应性肉芽肿性血管炎(CSS)是一种罕见的肉芽肿性坏死性小血管血管炎,其特征为存在哮喘、鼻窦炎和嗜酸性粒细胞增多。这种变应性血管炎和肉芽肿病的病因尚不清楚。其他常见表现包括肺部浸润、皮肤、胃肠道和心血管受累。尽管存在高球蛋白血症、IgE水平升高以及抗中性粒细胞胞浆抗体(40%呈阳性)表明存在自身免疫,但关于免疫复合物或细胞介导机制在该疾病中的作用尚无数据报道。我们报告一例27岁女性病例,该患者在长期(2002 - 2010年)服用全身性类固醇药物停药后不久,出现主要为下肢的疼痛性肿胀,并伴有广泛水疱和瘀斑以及发热。本病例报告的目的是指出在表现为伪装成史蒂文斯 - 约翰逊综合征/中毒性表皮坏死松解症(SJS/TENS)的广泛皮肤病变患者中存在CSS的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d47/3276904/3ff1bef864c0/IJD-56-718-g001.jpg

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