NEJM:抗MDA5皮肌炎的皮肤溃疡-病例报道

2019-08-01 xing.T MedSci原创

抗MDA5皮肌炎是与间质性肺病相关的皮肌炎的变种,其可快速发展。特征性皮肤症状包括Gottron丘疹的溃疡、口腔溃疡、手掌丘疹疼痛和脱发。给予免疫抑制和免疫调节疗法(包括羟氯喹、全身性糖皮质激素、霉酚酸酯和静脉注射免疫球蛋白),以及改善手部血流量的治疗(阿司匹林、己酮可可碱、硝苯地平、西地那非和肉毒杆菌毒素)。

患者为一名48岁的男子,因手部溃疡疼痛而到皮肤科就诊。最初症状出现在6个月前,并且与新发作的呼吸短促相关。

体检发现指尖和手指背部表面、肘部和口腔溃疡逐渐恶化以及眶周紫色皮疹。患者没有肌肉无力或关节炎。胸部计算机断层扫描(CT)显示不明确的结节,提示有组织性肺炎。患者接受了抗黑素瘤分化相关蛋白5(抗MDA5)皮肌炎的诊断,其在抗体检查中得到证实。结肠镜检查、腹部和胸部CT扫描以及前列腺特异性抗原检查没有癌症迹象。



抗MDA5皮肌炎是与间质性肺病相关的皮肌炎的变种,其可快速发展。特征性皮肤症状包括Gottron丘疹的溃疡、口腔溃疡、手掌丘疹疼痛和脱发。给予免疫抑制和免疫调节疗法(包括羟氯喹、全身性糖皮质激素、霉酚酸酯和静脉注射免疫球蛋白),以及改善手部血流量的治疗(阿司匹林、己酮可可碱、硝苯地平、西地那非和肉毒杆菌毒素)。

通过这种治疗方案,患者的手部溃疡在5个月内愈合,并且他的肺部症状也有所减轻。 
 
原始出处:

Alexandra Charrow,et al.Cutaneous Ulcerations in Anti-MDA5 Dermatomyositis.N Engl J Med 2019;https://www.nejm.org/doi/full/10.1056/NEJMicm1816147

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    2019-09-18 wetgdt
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    2019-08-01 旺医

    顶刊就是顶刊,谢谢梅斯带来这么高水平的研究报道,我们科里同事经常看梅斯,分享梅斯上的信息

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