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一名患有发作性血管性水肿和嗜酸性粒细胞增多症患者的腹水:跳出框框思考。

Ascites in a patient with episodic angio-oedema and eosinophilia: thinking outside the box.

作者信息

Rigopoulou Eirini I, Ioannou Maria, Papadamou Georgia, Dalekos Georgios N

机构信息

Department of Medicine and Research Laboratory of Internal Medicine, School of Medicine, University of Thessaly, Larissa, Greece.

Department of Pathology, School of Medicine, University of Thessaly, Larissa, Greece.

出版信息

BMJ Case Rep. 2017 Apr 21;2017:bcr-2017-219467. doi: 10.1136/bcr-2017-219467.

Abstract

Episodic angio-oedema with eosinophilia (EAE) or Gleich's syndrome is a rare condition characterised by recurrent episodes of oedema and eosinophilia, accompanied by urticaria, fever and weight gain. The presence of ascites has not been reported so far. We report a 21-year-old Caucasian woman who presented with marked ocular oedema and ascites. Laboratory evaluation revealed marked eosinophilia. During the last 3 months, three episodes of facial and neck oedema were reported, which resolved spontaneously over a period of 3-5 days. The diagnosis of EAE was established after exclusion of secondary causes (infections, allergic reactions, collagen diseases, neoplasms) and clonal disorders associated with marked eosinophilia. Low-dose steroids resulted in eosinophil decrease and complete resolution of symptoms, including ascites. This case highlights that ascites can be a very rare manifestation of EAE particularly if other more frequent causes of ascites have been excluded and the clinical and laboratory findings are supportive of EAE.

摘要

伴嗜酸性粒细胞增多的发作性血管性水肿(EAE)或格莱希综合征是一种罕见疾病,其特征为水肿和嗜酸性粒细胞增多反复发作,并伴有荨麻疹、发热和体重增加。目前尚未有腹水存在的报道。我们报告一名21岁的白种女性,她出现了明显的眼部水肿和腹水。实验室检查显示嗜酸性粒细胞显著增多。在过去3个月里,该患者报告了3次面部和颈部水肿发作,这些水肿在3 - 5天内自行消退。在排除继发原因(感染、过敏反应、胶原病、肿瘤)以及与显著嗜酸性粒细胞增多相关的克隆性疾病后,确诊为EAE。小剂量类固醇导致嗜酸性粒细胞减少,症状包括腹水完全消退。该病例突出表明,腹水可能是EAE非常罕见的一种表现,特别是在排除了其他更常见的腹水原因且临床和实验室检查结果支持EAE的情况下。

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