Maghrabi Yazid, Hakamy Sahar, Bahabri Nizar, AlSobaei Mohammed, Abuzinadah Ahmad R, Kurdi Maher
Neuromuscular Unit, King Abdulaziz University, Jeddah, Saudi Arabia.
Department of Medicine, Doctor Soliman Fakeeh Hospital, Jeddah, Saudi Arabia.
Neuromuscul Disord. 2021 Sep;31(9):881-885. doi: 10.1016/j.nmd.2021.07.001. Epub 2021 Jul 8.
Dermatomyositis (DM) is an immune-mediated inflammatory disease characterized by pathognomic lesions in skin and skeletal muscle including lymphocytic infiltrates. It rarely presents with ectopic lymphoid structures, as other autoimmune and chronic inflammatory diseases. We describe a case of a 47-year-old male, who presented clinically with proximal muscle weakness, skin rash and elevated creatin kinase (CK) levels. The muscle biopsy revealed inflammatory myopathy, with perifascicular pathology, and scattered ectopic lymphoid follicles-like structures harboring reactive B-cells. Clonality analysis of B-cells using polymerase chain reaction ruled out malignant lymphoma. The patient responded favorably to steroid therapy, and his muscle weakness improved. In conclusion, the clinical and histopathologic features of DM can be atypical, and the presence of lymphoid follicles, although rare, is not inevitably linked to an unfavorable prognosis.
皮肌炎（DM）是一种免疫介导的炎症性疾病，其特征为皮肤和骨骼肌出现特异性病变，包括淋巴细胞浸润。与其他自身免疫性和慢性炎症性疾病不同，它很少出现异位淋巴结构。我们报告一例47岁男性患者，临床表现为近端肌无力、皮疹和肌酸激酶（CK）水平升高。肌肉活检显示为炎症性肌病，伴有束周病理改变，并有散在的含有反应性B细胞的异位淋巴滤泡样结构。使用聚合酶链反应对B细胞进行克隆性分析排除了恶性淋巴瘤。患者对类固醇治疗反应良好，肌无力症状改善。总之，DM的临床和组织病理学特征可能不典型，淋巴滤泡的存在虽然罕见，但不一定与不良预后相关。
