The patient, a 69-year-old African American woman, presented with a three-month history of digital ulcers, Raynaud phenomenon, and occasional cough in addition to an eruption on her nose, back, and elbows. The patient denies any oral ulcers or nail changes. She also denied any photosensitivity, arthralgia, morning stiffness, muscle weakness or pain, fever, chills, weight loss, difficulty swallowing, hematuria, abdominal pain, headache, seizures, or mood disturbance. Mucocutaneous examination was remarkable for violaceous scaly plaques lining the nasal rim and on the elbows and back and multiple few-millimeter-sized, well-defined, grouped, eroded, and ulcerated violaceous papules overlying the joints on the palmar (palmar papules or inverse gottron papules)¹ and volar aspects of the hands. Muscle strength was within normal limits. Laboratory evaluation revealed a positive anti–melanoma differentiation antigen (MDA)-5 antibody in the setting of unremarkable antinuclear antibody, antineutrophil cytoplasmic antibody, double-stranded DNA, anti-sjogren syndrome A, anti-sjogren syndrome B, cyclic citrullinated peptide, creatinine kinase, uric acid, and complement levels. Erythrocyte sedimentation rate and C-reactive protein showed mild elevation. Skin biopsy from the elbow showed interface dermatitis, whereas that from digits showed infarct. Computed tomography) of the chest showed basilar predominant irregular consolidation with some reticulation and ground-glass attenuation, and a pulmonary function test showed a restrictive pattern. In view of pulmonary involvement, prominent vasculopathy, and to prevent rapid progressions, she was treated with 2 g/kg intravenous immunoglobulin every 28 days for 6 months and mycophenolic acid, prednisone, and nifedipine. The nasal rim sign has not been discussed or documented previously in the literature. However, on extensive literature search of clinical images of dermatomyositis, we came across some suggestions of similar involvement in other patients with anti-MDA-5 dermatomyositis.^2-4 Whether or not this sign is suggestive or specific for this antibody and its prognostic implication and association with airway/lung involvement remains to be established. In one review, the authors noted 4 of 10 patients who were anti-MDA-5 positive reported tender gums and/or oral erosions, significantly more than the group that was anti-MDA5 negative.¹ The nasal rim sign could possibly indicate another orifical predilection. Detailed documentation of clinical features and prognosis in further reports would provide more insight into the association.

该患者是一名 69 岁的非裔美国女性，有三个月的指部溃疡病史、雷诺现象和偶尔咳嗽，此外鼻子、背部和肘部也出现皮疹。患者否认有任何口腔溃疡或指甲变化。她还否认有任何光过敏、关节痛、晨僵、肌肉无力或疼痛、发烧、发冷、体重减轻、吞咽困难、血尿、腹痛、头痛、癫痫发作或情绪障碍。皮肤粘膜检查发现鼻缘、肘部和背部有紫罗兰色鳞状斑块，以及覆盖手掌关节的多个几毫米大小、边界清楚、成群、糜烂和溃疡的紫罗兰色丘疹（手掌丘疹或逆丘疹）丘疹）¹、手的掌侧。肌肉力量在正常范围内。实验室评估显示抗黑色素瘤分化抗原（MDA）-5抗体呈阳性，抗核抗体、抗中性粒细胞胞浆抗体、双链DNA、抗干燥综合征A、抗干燥综合征B、环瓜氨酸肽、肌酐无异常激酶、尿酸和补体水平。红细胞沉降率和C反应蛋白轻度升高。肘部皮肤活检显示界面皮炎，而手指活检显示梗死。胸部计算机断层扫描显示基底为主的不规则实变，伴有一些网状和毛玻璃状衰减，肺功能测试显示限制性模式。鉴于肺部受累、血管病变明显，并为了防止病情快速进展，每28天静脉注射2g/kg免疫球蛋白，持续6个月，并给予霉酚酸、泼尼松和硝苯地平治疗。先前的文献中尚未讨论或记录鼻缘征。然而，在对皮肌炎临床图像的广泛文献检索中，我们发现其他抗 MDA-5 皮肌炎患者也有类似的症状。^2-4该体征是否对该抗体具有提示性或特异性，及其预后意义以及与气道/肺部受累的关联仍有待确定。在一篇综述中，作者指出，10 名抗 MDA-5 阳性患者中有 4 名报告牙龈触痛和/或口腔糜烂，明显多于抗 MDA5 阴性患者。¹鼻缘征可能表明另一种口腔偏好。进一步报告中详细记录临床特征和预后将有助于更深入地了解这种关联。