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Degos-Like Cutaneous Findings in an Adult Woman With NXP-2-Associated Dermatomyositis.
JAMA Dermatology ( IF 10.9 ) Pub Date : 2019-12-26 , DOI: 10.1001/jamadermatol.2019.3940
Serena Giacalone 1 , Chiara Moltrasio 1 , Gianluca Nazzaro 1
Affiliation  

A woman in her 20s was admitted to the intensive care unit of our hospital owing to the onset of severe dysphagia that required a nasogastric tube. Her symptoms had started 2 months previously with progressive myalgia, proximal muscle weakness, and fever followed by diffuse edema involving the face and extremities. Laboratory studies showed a peak creatine kinase level of 12 410 U/L (normal range, 26-240 U/L; to convert to microkatals per liter, multiply by 0.0167), an erythrocyte sedimentation rate of 66 (normal range, 1-20 mm/h), and a D-dimer level of 1639 μg/mL (normal range, <0.5 μg/mL; to convert to nanomoles per liter, multiply by 5.476). A screening autoimmune disease panel revealed positive results for antinuclear antibody (titer, 1:320; speckled granular pattern) and an anticardiolipin antibody level of 14 U/mL (normal range, <10 U/mL). Electromyography showed widespread acute muscle damage; deltoid muscle biopsy findings were consistent with necrotizing myopathy. Suspecting an autoimmune myopathy, we treated the patient with 4 sessions of γ-globulin intravenously (2 g/kg over a 5-day period) followed by oral prednisolone (65 mg/d) and mycophenolate mofetil (1000 mg twice a day), which had a partial benefit to her clinical conditions. After 2 months, she noticed worsening of her weakness and facial edema. In addition, she developed a violaceous eruption of the eyelids associated with small white spots on her face, chest, and arms.



中文翻译:

与NXP-2相关的皮肌炎的成年女性的Degos皮肤病学发现。

一名20多岁的妇女因严重吞咽困难发作而需要进入鼻胃管,因此被送入我们医院的重症监护室。她的症状在2个月前开始,伴有进行性肌痛,近端肌无力和发烧,然后出现面部和四肢弥漫性水肿。实验室研究表明,肌酸激酶的峰值水平为12 410 U / L(正常范围为26-240 U / L;转换为微卡每升,乘以0.0167),红细胞沉降率为66(正常范围为1-20)毫米/小时),D-二聚体水平为1639μg/ mL(正常范围,<0.5μg/ mL;要转换为每升纳摩尔,乘以5.476)。筛查自身免疫性疾病小组显示抗核抗体(滴度为1:320;有斑点的颗粒状模式)的阳性结果和抗心磷脂抗体水平为14 U / mL(正常范围< 10 U / mL)。肌电图显示广泛的急性肌肉损伤。三角肌活检结果与坏死性肌病一致。怀疑自身免疫性肌病,我们对患者进行了4次γ-球蛋白静脉内治疗(5天期间2 g / kg),然后口服泼尼松龙(65 mg / d)和霉酚酸酯(每天两次1000 mg),这对她的临床状况有部分好处。2个月后,她注意到自己的无力和面部浮肿加重。另外,她的脸,胸部和手臂上的小白斑也引起了眼睑的紫罗兰性喷发。我们对患者进行了4次γ-球蛋白静脉内治疗(5天2 g / kg),然后口服泼尼松龙(65 mg / d)和霉酚酸酯(每天两次1000 mg)治疗,这部分受益适应她的临床情况。2个月后,她注意到自己的无力和面部浮肿加重。另外,她的脸,胸部和手臂上的小白斑也引起了眼睑的紫罗兰性喷发。我们对患者进行了4次γ-球蛋白静脉内治疗(5天期间2 g / kg),然后口服泼尼松龙(65 mg / d)和霉酚酸酯(每天两次1000 mg)治疗,这部分受益适应她的临床情况。2个月后,她注意到自己的无力和面部浮肿加重。另外,她的脸,胸部和手臂上的小白斑也引起了眼睑的紫罗兰性喷发。

更新日期:2020-02-12
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