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伴有弥散性血管内凝血(DIC)的肼屈嗪诱导的抗中性粒细胞胞质抗体(ANCA)血管炎的严重皮肤表现,无肾或肺受累。

Severe cutaneous presentation of hydralazine-induced ANCA vasculitis without renal or pulmonary involvement, complicated by DIC.

机构信息

Internal Medicine, Albany Medical Center, Albany, New York, USA

Internal Medicine, Albany Medical Center, Albany, New York, USA.

出版信息

BMJ Case Rep. 2021 May 11;14(5):e238609. doi: 10.1136/bcr-2020-238609.

Abstract

Hydralazine is a common arterial vasodilator used in the management of congestive heart failure and hypertension. It can be associated with drug-induced lupus and less commonly antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Drug-induced AAV typically has a favourable long-term prognosis. It is not commonly associated with primary skin involvement, as most cases also have notable kidney and lung disease. Cases with isolated skin findings are rare. We present a rare case of a 60-year-old woman on long-term hydralazine who presented with AAV with primary skin and mucosal involvement, in the form of diffuse bullous and ulcerative lesions, which posed a diagnostic challenge. Her hospital course was marked by several complications including disseminated intravascular coagulation. She required intensive therapy with high-dose steroids, plasmapheresis and rituximab. She tolerated immunosuppression well and with multidisciplinary supportive care, she recovered well and was able to be discharged from the hospital.

摘要

肼屈嗪是一种常用的动脉血管扩张剂,用于治疗充血性心力衰竭和高血压。它可引起药物诱导性狼疮,且不太常见的情况下会引起抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)。药物诱导性 AAV 通常具有良好的长期预后。它通常不与原发性皮肤受累相关,因为大多数病例还伴有明显的肾脏和肺部疾病。孤立性皮肤表现的病例很少见。我们报告了一例罕见的长期使用肼屈嗪的 60 岁女性,她患有 AAV,表现为弥漫性大疱性和溃疡性病变,这对诊断构成了挑战。她的住院过程中出现了多种并发症,包括弥散性血管内凝血。她需要接受大剂量类固醇、血浆置换和利妥昔单抗的强化治疗。她对免疫抑制治疗耐受良好,在多学科支持治疗下,她恢复良好并能够出院。

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