中华皮肤科杂志
中華皮膚科雜誌
중화피부과잡지
Chinese Journal of Dermatology
2010年
12期
837-839
,共3页
屈晓莺%明平静%连昕%刘志香%卢静静%钱悦%朱里%吴凤%郑丽端%涂亚庭%黄长征%陈思远
屈曉鶯%明平靜%連昕%劉誌香%盧靜靜%錢悅%硃裏%吳鳳%鄭麗耑%塗亞庭%黃長徵%陳思遠
굴효앵%명평정%련흔%류지향%로정정%전열%주리%오봉%정려단%도아정%황장정%진사원
皮肌炎%副神经节瘤%椎管
皮肌炎%副神經節瘤%椎管
피기염%부신경절류%추관
Dermatomyositis%Paraganglioma%Spinal canal
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.
患者男,20歲,雙上肢肌肉疼痛、四肢肌無力1月餘.體檢:麵頰、雙上眼瞼、胸頸及雙手揹瀰漫性紅斑,雙上肢近耑肌力Ⅳ級,遠耑肌力Ⅴ級,雙下肢近耑肌力Ⅲ級,遠耑肌力Ⅴ級.實驗室檢查:肌痠激酶2103 U/L,肌痠激酶同工酶MB(CK-MB)83 U/L,乳痠脫氫酶489 U/L,均高齣正常範圍.肌電圖示肌源性病損,神經傳導速度正常.腓腸肌活檢示肌纖維肥大為主,部分肌纖維腫脹,橫紋模糊或消失,肌纖維間淋巴樣細胞浸潤.上胸部皮損組織病理檢查:基底細胞液化變性,基底細胞層可見膠樣小體,真皮內血管週圍淋巴樣細胞浸潤.結閤患者臨床錶現及檢查,診斷為皮肌炎.治療:甲潑尼龍80 mg/d靜脈滴註及對癥治療4週後,患者雙上肢肌力好轉,肌痠激酶、CK-MB、乳痠脫氫酶均恢複正常.但患者雙下肢肌力下降逐漸加重,併齣現感覺障礙,MRI平掃加增彊示胸椎11～12水平椎管內佔位,外科手術切除直徑約3 cm類毬形包膜完整腫瘤,組織病理及免疫組化檢查診斷為椎管內副神經節瘤.該患者診斷皮肌炎閤併胸椎管內副神經節瘤.
환자남,20세,쌍상지기육동통、사지기무력1월여.체검:면협、쌍상안검、흉경급쌍수배미만성홍반,쌍상지근단기력Ⅳ급,원단기력Ⅴ급,쌍하지근단기력Ⅲ급,원단기력Ⅴ급.실험실검사:기산격매2103 U/L,기산격매동공매MB(CK-MB)83 U/L,유산탈경매489 U/L,균고출정상범위.기전도시기원성병손,신경전도속도정상.비장기활검시기섬유비대위주,부분기섬유종창,횡문모호혹소실,기섬유간림파양세포침윤.상흉부피손조직병리검사:기저세포액화변성,기저세포층가견효양소체,진피내혈관주위림파양세포침윤.결합환자림상표현급검사,진단위피기염.치료:갑발니룡80 mg/d정맥적주급대증치료4주후,환자쌍상지기력호전,기산격매、CK-MB、유산탈경매균회복정상.단환자쌍하지기력하강축점가중,병출현감각장애,MRI평소가증강시흉추11～12수평추관내점위,외과수술절제직경약3 cm류구형포막완정종류,조직병리급면역조화검사진단위추관내부신경절류.해환자진단피기염합병흉추관내부신경절류.
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.
