一名53岁的男子在左上臂出现难治性脂膜炎,持续了10个月。病人被诊断为狼疮,其中开始口服糖皮质激素治疗。四个月前,在同一区域观察到溃疡。相反,Dapson被管理,瘢痕溃疡,但扩大脂膜炎。五周前,他发烧了,生产性咳嗽,和呼吸困难.三周前,额头上有皮疹,左耳廓在脖子后面,和左肘的伸肌方面。胸部计算机断层扫描显示右肺肺炎,之后,患者的呼吸困难恶化。患者入院并根据皮肤发现诊断为抗MDA5抗体阳性的肌病性皮肌炎(ADM)。高铁蛋白血症,和快速进展的弥漫性肺部阴影。糖皮质激素脉冲治疗,静脉注射环磷酰胺,他克莫司开始了,后来,联合血浆置换治疗。然而,他的病情恶化,需要体外膜氧合治疗。患者在住院后第28天死亡。尸检显示透明质化至弥漫性肺泡损伤的纤维化阶段。3份皮肤活检标本从最初发病时开始观察到粘液病毒抗性蛋白A的强表达,与ADM一致。抗MDA5抗体阳性ADM不仅表现出典型的皮肤症状,但也很少发生局部脂膜炎,例如在目前的情况下。在病因不明的脂膜炎患者中,鉴别诊断应包括ADM初始症状的可能性.
A 53-year-old man was presented with refractory panniculitis on the left upper arm that had persisted for 10 months. The patient was diagnosed with lupus profundus, wherein oral glucocorticoid therapy was initiated. Four months prior, ulceration was observed in the same area. Dapson was administered instead, scarring the ulcer but enlarging the panniculitis. Five weeks earlier, he developed a fever, productive cough, and dyspnoea. Three weeks earlier, a skin rash was observed on the forehead, left auricle posterior to the neck, and extensor aspect of the left elbow. Chest computed tomography showed pneumonia in the right lung, after which the patient\'s dyspnoea worsened. The patient was admitted and diagnosed with anti-MDA5 antibody-positive amyopathic dermatomyositis (ADM) based on skin findings, hyperferritinaemia, and rapidly progressive diffuse lung shadows. Glucocorticoid pulse therapy, intravenous cyclophosphamide, and tacrolimus were initiated, and later, plasma exchange therapy was combined. However, his condition worsened and required management with extracorporeal membrane oxygenation. The patient expired on day 28 after hospitalisation. An autopsy revealed hyalinising to fibrotic stages of diffuse alveolar damage. Strong expression of myxovirus resistance protein A was observed in three skin biopsy specimens from the time of initial onset, consistent with ADM. Anti-MDA5 antibody-positive ADM not only manifests typical cutaneous symptoms, but also rarely occurs with localised panniculitis, such as in the present case. In patients with panniculitis of unknown aetiology, the possibility of initial symptoms of ADM should be included in the differential diagnosis.