文献简介

出版社:JAMA Dermatology

作  者:Emily M. Erstine,MD, MBA; Angela M. B. Collie,MD, PhD; Melissa P. Piliang, MD

编  号:

关键字:Granulomatosis with polyangiitis

年  份:2016   点击量:166

文献摘要 全文翻译

A man in his 60s who was a former smoker with a medical history of chronic obstructive pulmonary disease, type 2 diabetes, chronic kidney disease, anemia, and cervical myelopathy experienced low-grade fevers, unintentional 80-pound weight loss, productive cough, and development of cutaneous ulcers over a 6-month period after cervical spine fusion. Two ulcers developed at the surgical site, and 2 similar ulcers developed on his back shortly after cyst excision. He had been recently diagnosed with lung cancer based on computed tomographic (CT) and positron emission tomographic imaging showing an enhancing, hypermetabolic, 7-cm cavitary right upper lobe mass with mediastinal adenopathy. A chest x-ray 6 months prior revealed negative results. A few days after establishing care, he presented to the emergency department with extreme weakness and intolerable pain related to his ulcers. Physical examination revealed an ill-appearing man. In the right upper lobe, there were decreased breath sounds with clear auscultation in the remaining chest. On the right anterior neck were two, 3- to 4-cm tender ulcers extending to the deep subcutaneous fat with erythematous, friable borders (Figure). On his back were 2 similar 2- to 2.5-cm ulcers. Inflammatory markers were notably elevated. A complete autoimmune panel, indirect immunofluorescence assays, enzyme immunoassays for antineutrophil cytoplasmic antibodies(c-ANCA)/proteinase 3, antineutrophil cytoplasmic antibody/ myeloperoxidase, a quantifier on gold test, cultures of  blood, broncheoalveolar washings, and tissue analysis were performed. Only c-ANCA testing revealed positive results. A CT scan of the sinus showed multiple bony defects and acute and chronic sinusitis. A transbronchial biopsy of the lung and excisional biopsy of the skin bridge between back ulcers were performed.

患者男性,60余岁,有吸烟史,有慢性阻塞性肺疾病、2型糖尿病、慢性肾病、贫血,脊髓型颈椎病史,出现过低烧、体重意外减少80磅、排痰性咳嗽,且颈椎融合术后6个月皮肤溃疡发展。手术部位出现两个溃疡,囊肿切除后不久背部出现2个类似溃疡。近期患者根据计算机断层扫描(CT)被诊断为肺癌,正电子发射体层成像示右上叶7cm空洞增强,代谢率高伴纵膈淋巴结肿大。6个月前胸部X线检查结果呈阴性。接受几天治疗后,患者向急诊科陈述其极端虚弱,且溃疡处疼痛难耐。体格检查患者呈病态。右上叶呼吸音减低与其余胸部听诊音清晰。右颈前侧可见2个3至4厘米的疼痛溃疡,且溃疡延伸至深层皮下脂肪,边界发红(图)。患者背部有2个类似的2至2.5厘米的溃疡,炎性显著升高。行完全免疫组检查、间接免疫荧光检查、抗中性粒细胞胞浆抗体酶免疫测定(c-ANCA)/蛋白酶3、抗中性粒细胞胞浆抗体/髓过氧化物酶检查、金含量试验、血液培养、支气管肺泡清洗和组织分析。仅有c-ANCA测试结果为阳性。静脉窦CT扫描示多发性骨缺损及急慢性鼻窦炎。行经支气管肺部活检,以及皮肤和背部溃疡切除活检。