[NEJM临床医学影像]：皮肌炎的眶周水肿

A 61-year-old woman presented to the rheumatology clinic with a 6-month history of periorbital swelling and shoulder weakness. She reported no dyspnea, cough, or joint pain. The physical examination was notable for erythema of the upper and lower eyelids and substantial periorbital edema (Panel A). There was also midfacial erythema involving the nasolabial folds, poikiloderma of the upper back, and 4/5 strength of the shoulder abductors on both sides. Laboratory testing showed a creatine kinase level of 6300 U per liter (reference range, 24 to 170), an antinuclear antibody titer of 1:320, and positivity for anti-p155/140 myositis autoantibody. Magnetic resonance imaging of the right deltoid muscle showed muscle edema. A skin biopsy showed interface dermatitis. A diagnosis of dermatomyositis was made. Dermatomyositis is an idiopathic inflammatory myopathy characterized by immune-mediated muscle and skin injury. As in this case, the pathognomic skin finding of a periorbital heliotrope rash may be accompanied by eyelid edema in dermatomyositis. Other typical skin findings in this patient included shawl sign and a malar rash that did not spare the nasolabial folds. Treatment with oral glucocorticoids, methotrexate, and intravenous immune globulin was initiated. Screening for cancer was negative. At the 2-month follow-up, the patient’s weakness, rashes, and periorbital edema had abated (Panel B).

一名61岁女性因眶周肿胀和肩部无力6个月到风湿病门诊就诊。患者自诉无呼吸困难、咳嗽或关节痛。体格检查发现上、下眼睑红斑和明显的眶周水肿(图A)。还有涉及鼻唇沟的面中部红斑，上背部皮肤异色，双侧肩外展肌肌力4/5。实验室检查显示肌酸激酶水平为6,300 U / l(参考范围，24 ~ 170 U / l)，抗核抗体滴度为1∶320，抗p155/140肌炎自身抗体阳性。右三角肌磁共振成像示肌肉水肿。皮肤活检示界面性皮炎。诊断为皮肌炎。皮肌炎是一种以免疫介导的肌肉和皮肤损伤为特征的特发性炎症性肌病。与此例一样，皮肌炎的特征性皮肤表现眶周向阳样皮疹可伴有眼睑水肿。该患者的其他典型皮肤表现包括披肩征和覆盖鼻唇沟的颧部皮疹。开始口服糖皮质激素、甲氨蝶呤和静脉注射免疫球蛋白治疗。癌症筛查结果为阴性。2个月随访时，患者的乏力、皮疹和眶周水肿减轻(图B)。