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一种伴有抗中性粒细胞胞浆抗体和抗肾小球基底膜抗体的 Churg-Strauss 综合征样特征的血管炎不典型皮肤表现。

An atypical cutaneous presentation of vasculitis with features of Churg-Strauss syndrome, associated with anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane antibodies.

机构信息

Department of Dermatology, Chelsea and Westminster NHS Foundation Trust, London, UK.

出版信息

Clin Exp Dermatol. 2009 Dec;34(8):e577-80. doi: 10.1111/j.1365-2230.2009.03248.x. Epub 2009 Jun 24.

DOI:10.1111/j.1365-2230.2009.03248.x
PMID:19558532
Abstract

We report the case of a 59-year-old woman who presented with a persistent papular and nodular cutaneous eruption and new-onset asthma, with normal renal function but persistent haematuria and proteinuria. Investigations revealed eosinophilia, both antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies on serological testing (double-positive vasculitis), and a focal necrotizing glomerulonephritis on renal biopsy. Histological examination of a skin biopsy showed a dense neutrophilic infiltrate with focal fibrinoid necrosis and few eosinophils. The clinical and pathological features suggested a double-positive vasculitis/Churg-Strauss overlap syndrome presenting with a predominantly neutrophilic dermatosis. Specific cutaneous features in patients with double-positive vasculitis have not been documented previously. The patient has responded extremely well to immunosuppressive treatment and her disease is currently in remission.

摘要

我们报告了一例 59 岁女性患者,其表现为持续的丘疹性和结节性皮肤疹和新发哮喘,肾功能正常,但持续血尿和蛋白尿。检查发现嗜酸性粒细胞增多,血清学检查发现抗中性粒细胞胞质抗体和抗肾小球基底膜抗体均为阳性(双阳性血管炎),肾活检显示局灶坏死性肾小球肾炎。皮肤活检的组织学检查显示密集的中性粒细胞浸润,伴有局灶性纤维蛋白样坏死和少量嗜酸性粒细胞。临床和病理特征提示双阳性血管炎/Churg-Strauss 重叠综合征,表现为主要为中性粒细胞性皮肤病。以前没有记录过双阳性血管炎患者的特定皮肤特征。该患者对免疫抑制治疗反应非常好,目前疾病处于缓解期。

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引用本文的文献

1
Cutaneous manifestations of Churg-Strauss syndrome: report of two cases and review of the literature.变应性肉芽肿性血管炎(Churg-Strauss 综合征)的皮肤表现:两例报告及文献复习。
Clin Rheumatol. 2011 Apr;30(4):573-80. doi: 10.1007/s10067-010-1593-1. Epub 2010 Oct 15.