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白细胞破碎性血管炎:诊断困境的描述

Leukocytoclastic Vasculitis: Depiction of the Diagnostic Dilemma.

作者信息

Bhesania Siddharth, Raol Karanrajsinh, Medina Chanoa, Ilyas Sahar, Bhesania Janki, Barmanwalla Alina

机构信息

Internal Medicine, Overlook Medical Center, Summit, USA.

Internal Medicine, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, USA.

出版信息

Cureus. 2021 Aug 26;13(8):e17462. doi: 10.7759/cureus.17462. eCollection 2021 Aug.

Abstract

Vasculitis is classified based on the size of the blood vessels involved. Sub-group Leukocytoclastic vasculitis (LCV) refers to small blood vessel inflammation, which involves cutaneous capillaries and venules. To date, there have been myriad primary and secondary probable causes of LCV. Here, we present a case of an 86-year-old male who presented with non-blanchable purpura involving the ankles, knees, and palms. The patient had idiopathic pulmonary fibrosis (IPF), for which he had been on long-term oxygen therapy and chronic corticosteroids. He was recently started on Bactrim DS (trimethoprim-sulfamethoxazole double strength) for prophylaxis of pneumocystis pneumonia. After a meticulous workup, including a skin biopsy, the causative agent of the LCV was established to be Bactrim DS, and the event was likely triggered by superimposed acute stress of sepsis secondary to UTI and bacteremia. There were several diagnostic dilemmas due to the ongoing chronic medical conditions; however, the occurrence of LCV while being on chronic corticosteroids was concerning as it should have prevented such an untoward occurrence. Eventually, the presentation subsided past an increase in the dose of corticosteroids and discontinuation of Bactrim DS. This raises concern regarding either the dose-dependent immunosuppressive effects of corticosteroids or deficits in our current understanding of the mechanism of action. Additionally, it necessitates further exploration into the causes of LCV and a thorough understanding of its pathogenesis.

摘要

血管炎是根据受累血管的大小进行分类的。亚组白细胞破碎性血管炎(LCV)指的是小血管炎症,累及皮肤毛细血管和小静脉。迄今为止,LCV有众多原发性和继发性可能病因。在此,我们报告一例86岁男性患者,其双踝、双膝及手掌出现不可压褪色性紫癜。该患者患有特发性肺纤维化(IPF),长期接受氧疗及慢性皮质类固醇治疗。他近期开始服用复方新诺明双倍剂量片(甲氧苄啶 - 磺胺甲恶唑双倍强度)以预防肺孢子菌肺炎。经过细致检查,包括皮肤活检,确定LCV的致病因素为复方新诺明双倍剂量片,该事件可能由尿路感染和菌血症继发的败血症叠加急性应激引发。由于存在持续的慢性疾病,出现了几个诊断难题;然而,在接受慢性皮质类固醇治疗期间发生LCV令人担忧,因为这本应预防此类不良事件的发生。最终,增加皮质类固醇剂量并停用复方新诺明双倍剂量片后,症状消退。这引发了对于皮质类固醇剂量依赖性免疫抑制作用或我们目前对作用机制理解不足的关注。此外,有必要进一步探究LCV的病因并深入了解其发病机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/346c/8478687/e9f4d5509362/cureus-0013-00000017462-i01.jpg

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