皮肌炎合并胸椎管内副神经节瘤一例  

Caraganglioma in thoracic vertebral canal superimposed on dermatomyositis： a case report

作　　者：屈晓莺[1] 明平静 连昕[1] 刘志香[1] 卢静静[1] 钱悦[1] 朱里[1] 吴凤[1] 郑丽端[2] 涂亚庭[1] 黄长征[1] 陈思远[1] 

机构地区：[1]华中科技大学同济医学院附属协和医院皮肤科,武汉430022 [2]华中科技大学同济医学院附属协和医院病理科,武汉430022 [3]解放军第457医院医务处

出　　处：《中华皮肤科杂志》2010年第12期837-839,共3页Chinese Journal of Dermatology

摘　　要：患者男,20岁,双上肢肌肉疼痛、四肢肌无力1月余.体检：面颊、双上眼睑、胸颈及双手背弥漫性红斑,双上肢近端肌力Ⅳ级,远端肌力Ⅴ级,双下肢近端肌力Ⅲ级,远端肌力Ⅴ级.实验室检查：肌酸激酶2103 U/L,肌酸激酶同工酶MB（CK-MB）83 U/L,乳酸脱氢酶489 U/L,均高出正常范围.肌电图示肌源性病损,神经传导速度正常.腓肠肌活检示肌纤维肥大为主,部分肌纤维肿胀,横纹模糊或消失,肌纤维间淋巴样细胞浸润.上胸部皮损组织病理检查：基底细胞液化变性,基底细胞层可见胶样小体,真皮内血管周围淋巴样细胞浸润.结合患者临床表现及检查,诊断为皮肌炎.治疗：甲泼尼龙80 mg/d静脉滴注及对症治疗4周后,患者双上肢肌力好转,肌酸激酶、CK-MB、乳酸脱氢酶均恢复正常.但患者双下肢肌力下降逐渐加重,并出现感觉障碍,MRI平扫加增强示胸椎11～12水平椎管内占位,外科手术切除直径约3 cm类球形包膜完整肿瘤,组织病理及免疫组化检查诊断为椎管内副神经节瘤.该患者诊断皮肌炎合并胸椎管内副神经节瘤.A 20-year-old male patient presented with myalgia of upper limbs and myasthenia of extremities for more than 1 month. Physical examination showed diffuse erythema on the cheeks, upper eyelids, upper chest, neck and dorsa of the hands. The myodynamia of the proximal and distal muscles of upper and lower extremities was grade Ⅳ, Ⅴ, Ⅲ and Ⅴ respectively. Laboratory examinations revealed that the serum levels of creatine kinase, CK-MB and lactate dehydrogenase were 2103 U/L, 83 U/L and 489 U/L respectively, which were all above the normal range. Electromyogram revealed myopathic abnormality and normal nerve conduction velocity. Histopathology of gastrocnemius muscle showed hypertrophy and swelling of muscle fibers, disappearance or fuzziness of transverse striation, and intermuscular lymphoid cell infiltration. A biopsy of the skin lesion from the upper chest showed liquefaction degeneration of and colloid bodies in basal cell layer, perivascular lymphoid cell infiltration in the dermis. A diagnosis of dermatomyositis was established based on the clinical and laboratory findings. After management with intravenous prednisolone 80 mg once daily and symptomatic treatment for 4 weeks, the myodynamia of upper limbs was improved, serum levels of creatine kinase,CK-MB and lactate dehydrogenase reached the normal ranges. However, the myodynamia of lower limbs progressively deteriorated with the emergence of paresthesia. Enhanced MRI scan showed a tumor in the vertebral canal at the level of thoracic vertebra 11 to 12. A spherical encapsulated tumor measuring 3 cm in diameter was surgically removed. The tumor was diagnosed as paraganglioma in vertebral canal according to pathological and immunohistochemical findings. The patient was finally diagnosed with paraganglioma in vertebral canal superimposed on dermatomyositis.

关 键 词：皮肌炎 副神经节瘤 椎管 

分 类 号：R593.26[医药卫生—内科学] R739.4[医药卫生—临床医学]