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一个家族中表现为短暂性周围关节炎的遗传性血管性水肿。

Hereditary angioedema in a family presenting as transient periarthritis.

作者信息

Adhikesavan Leena G, Olenginski Thomas P

机构信息

Department of Rheumatology, Geisinger Medical Center, Danville, PA-17822, USA.

出版信息

J Clin Rheumatol. 2008 Oct;14(5):289-91. doi: 10.1097/RHU.0b013e3181886ef9.

Abstract

Hereditary angioedema (HAE) is a rare condition known to cause episodic, self-limiting, nonpruritic, nonpitting edema that involves skin and visceral organs. It may affect any external body surface including face, extremities, and genitalia. Most commonly involved viscera are gastrointestinal and respiratory systems. Patients may have severe abdominal pain because of edema of the bowel wall. This disease can cause life threatening laryngeal edema if it involves the airway.We describe a patient with HAE who was initially diagnosed with arthritis after she had recurrent edema around her peripheral joints. Diagnosis of HAE in her led to the same diagnosis in her sister and her father. HAE should be considered in the differential diagnosis of recurrent attacks of periarticular swelling.

摘要

遗传性血管性水肿(HAE)是一种罕见疾病,已知可引起发作性、自限性、无瘙痒、非凹陷性水肿,累及皮肤和内脏器官。它可影响任何体表部位,包括面部、四肢和生殖器。最常累及的内脏是胃肠道和呼吸系统。患者可能因肠壁水肿而出现严重腹痛。如果累及气道,这种疾病可导致危及生命的喉水肿。我们描述了一名HAE患者,她最初在外周关节反复出现水肿后被诊断为关节炎。对她的HAE诊断导致她的姐姐和父亲也被诊断出同样的疾病。在鉴别诊断关节周围肿胀反复发作时应考虑HAE。

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