Department of Dermatovenereology, the First Affiliated Hospital of Anhui Medical University, Hefei, Anhui, China.
Medicine (Baltimore). 2023 Dec 15;102(50):e36234. doi: 10.1097/MD.0000000000036234.
Patients who have myasthenia gravis or dermatomyositis show clinical signs of muscular weakening. Ocular muscle involvement is uncommon, and symmetrical proximal limb weakness is the typical presentation of dermatomyositis. However, the earliest and most noticeable sign in those with myasthenia gravis is extraocular muscular paralysis. Dermatomyositis is frequently complicated by malignancy, and the common malignancies associated with dermatomyositis vary by region and ethnicity, while thymoma is relatively rare. About 10% to 15% of people with myasthenia gravis have thymoma, which is involved in the etiology of the disease.
A 68-year-old female presented with ocular muscle weakness for 10 days that manifested as bilateral blepharoptosis with the phenomenon of "light in the morning and heavy in the evening." Imaging examination showed anterior mediastinal thymic tumor with metastasis.
After a thorough physical examination, we discovered bilateral upper limbs with grade IV muscle strength and the typical rash of dermatomyositis. In combination with elevated serum kinase levels and electromyography suggesting myogenic damage, the patient was finally diagnosed as dermatomyositis with multiple metastases of thymoma.
The patient received oral hydroxychloroquine sulfate, topical corticosteroids, and tacrolimus ointment, but these did not work very well. Subsequently, the patient underwent surgery combined with radiotherapy for the thymoma.
Muscle weakness in the patient improved after effective treatment of tumor, and the rash mostly disappeared.
Ocular muscle weakness and thymoma are more common in myasthenia gravis, but we cannot ignore the possibility of dermatomyositis. To further establish the diagnosis, a thorough physical examination and laboratory findings are required. Further tumor screening should be performed for patients with dermatomyositis. Early detection and management of possible tumors are essential to the treatment of dermatomyositis linked to malignancies.
患有重症肌无力或皮肌炎的患者会出现肌肉无力的临床症状。眼外肌受累并不常见，皮肌炎的典型表现为对称性近端肢体无力。然而，重症肌无力患者最早和最明显的体征是眼外肌瘫痪。皮肌炎常并发恶性肿瘤，与皮肌炎相关的常见恶性肿瘤因地区和种族而异，而胸腺瘤相对少见。约 10%至 15%的重症肌无力患者伴有胸腺瘤，胸腺瘤参与了疾病的发病机制。
一名 68 岁女性因眼部肌肉无力就诊 10 天，表现为双侧眼睑下垂，伴有“晨轻暮重”现象。影像学检查显示前纵隔胸腺肿瘤伴转移。
经过详细的体格检查，我们发现患者双侧上肢肌力为四级，且具有皮肌炎的典型皮疹。结合血清激酶水平升高和肌电图提示肌源性损伤，最终诊断为皮肌炎伴胸腺瘤多处转移。
患者接受了羟氯喹硫酸盐口服、局部皮质类固醇和他克莫司软膏治疗，但效果不佳。随后，患者接受了手术联合放疗治疗胸腺瘤。
患者的肌肉无力在肿瘤得到有效治疗后得到改善，皮疹大部分消退。
眼外肌无力和胸腺瘤在重症肌无力中更为常见，但我们不能忽视皮肌炎的可能性。为了进一步明确诊断，需要进行全面的体格检查和实验室检查。对于皮肌炎患者，应进一步进行肿瘤筛查。早期发现和管理可能的肿瘤对于治疗与恶性肿瘤相关的皮肌炎至关重要。
