Department of Medicine, Albert Einstein College of Medicine, Bronx, New York, USA
Department of Medicine, Albert Einstein College of Medicine, Bronx, New York, USA.
BMJ Case Rep. 2024 Mar 21;17(3):e253960. doi: 10.1136/bcr-2022-253960.
Antimelanoma differentiation-associated protein 5 positive dermatomyositis (MDA5 DM) is a rare subtype of idiopathic inflammatory myopathy. There are limited data available regarding the cutaneous manifestations of MDA5 DM in the African American population. We presented the case of a male patient in his early 20s who presented with debilitating cutaneous ulceration and myopathy. Workup revealed interstitial lung disease (ILD) and positive MDA5 serology consistent with MDA5 DM. He made a remarkable recovery in terms of myopathy and cutaneous ulcerations with a multipronged regimen of prednisone, intravenous immunoglobulin and mycophenolate mofetil. However, there was a progression of ILD on this regimen which warranted use of rituximab.
抗黑色素瘤分化相关蛋白 5 阳性皮肌炎（MDA5 DM）是一种罕见的特发性炎症性肌病亚型。关于非裔美国人中 MDA5 DM 的皮肤表现，相关数据有限。我们报告了一名 20 多岁的男性患者，他表现为进行性皮肤溃疡和肌病。检查发现间质性肺病（ILD）和 MDA5 血清学阳性，符合 MDA5 DM 的诊断。他通过泼尼松、静脉注射免疫球蛋白和霉酚酸酯的多管齐下的治疗方案，在肌病和皮肤溃疡方面取得了显著的恢复。然而，ILD 在此治疗方案下仍有进展，因此需要使用利妥昔单抗。
