Abstract：
Buerger\'s disease is characterized by peripheral ischemia due to occlusion of small- and medium-sized arteries in the extremities. This report describes a case of Buerger\'s disease in a 51-year-old male who presented with findings resembling systemic sclerosis. The patient exhibited Raynaud\'s phenomenon in year X-3, which developed to skin hardening, nail avulsion, and ulceration of the right fingers in year X. Diagnostic testing showed positive microvasculopathy on nailfold videocapillaroscopy (NVC) and positive fibrosis on skin biopsy. Although the patient fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for systemic sclerosis, several findings in this case were atypical for systemic sclerosis, including left-right asymmetry in finger involvement, nail loss, and negative autoantibody tests. Contrast-enhanced computed tomography showed poor perfusion of the right ulnar artery, and a heavy smoking history was established in the patient case. Therefore, based on Shionoya\'s criteria, he was diagnosed with a case of Buerger\'s disease confined to the upper extremity. Smoking cessation and vasodilator therapy resulted in the prompt resolution of ischemic symptoms, skin hardening, and ulcerations. Furthermore, NVC abnormalities improved, and ulnar artery occlusion showed reperfusion on repeat testing. The present case suggests that hypoxemia-driven microvasculopathy may contribute to vascular occlusion and skin fibrosis observed in this atypical presentation.
摘要：
Buerger病的特征是由于四肢中小动脉阻塞引起的外周缺血。本报告描述了一名51岁男性的Buerger病病例，该病例表现出类似于系统性硬化症的发现。患者在X-3年表现出雷诺现象，发展为皮肤硬化，指甲撕脱,X年的右手手指溃疡。诊断测试显示，甲叠录影带镜（NVC）显示微血管病变阳性，皮肤活检显示纤维化阳性。尽管患者符合2013年美国风湿病学会/欧洲抗风湿病联盟（ACR/EULAR）系统性硬化症的分类标准，在这种情况下，一些发现是不典型的系统性硬化症，包括手指参与的左右不对称，指甲脱落,自身抗体检测阴性.对比增强计算机断层扫描显示右尺动脉灌注不良，并在患者病例中建立了大量吸烟史。因此,根据Shionoya的标准，他被诊断出患有局限于上肢的Buerger病。戒烟和血管扩张剂治疗可迅速缓解缺血症状，皮肤硬化,和溃疡。此外，NVC异常改善，重复测试显示尺动脉闭塞再灌注。本病例表明，低氧血症驱动的微血管病变可能导致在这种非典型表现中观察到的血管闭塞和皮肤纤维化。
