中华皮肤科杂志 ›› 2006, Vol. 39 ›› Issue (1): 44-46.

• 论著 • 上一篇    下一篇

以肌病为首发表现的系统性淀粉样变性一例

李希清, 席丽艳, 刘红芳, 李伯友, 张军民, 林宝珠   

  1. 中山大学附属第二医院皮肤科 广州 510120
  • 收稿日期:2005-08-01 发布日期:2006-01-15

Systemic amyloidosis with myopathy as the first manifestation:a case report

LI Xi-qing, XI Li-yan, LIU Hong-fang, LI Bo-you, ZHANG Jun-min, LIN Bao-zhu   

  1. Department of Dermatology, The Second Affiliated Hospital, SUN Yat-Sen University, Guangzhou 510120, China
  • Received:2005-08-01 Published:2006-01-15

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摘要： 目的 探讨系统性淀粉样变性的临床病理特点,鉴别诊断,治疗与预后.方法 分析我科收治的1例以肌肉表现为首发症状的系统性淀粉样变性的临床、病理、实验室检查特点,并对文献进行复习.结果 该患者以全身肌肉变硬、巨舌为首发症状,检查进一步发现心血管系统、呼吸系统、消化系统、肾脏受累.经皮肤、肌肉、胃肠黏膜组织病理H-E染色、刚果红染色、偏振光显微镜检查证实为系统性淀粉样变性.结论 系统性淀粉样变性可以肌肉病变为首发表现.

关键词: 淀粉样变性, 肌疾病

Abstract: Objective To elucidate the clinicopathological characteristic,differential diagnosis,treatment and prognosis of systemic amyloidosis.Methods An inpatient diagnosed as systemic amyloidosis was analyzed for clinical and pathological features as well as laboratory findings.The related literature was reviewed.Results The patient was confirmed to have amyloidosis of the muscle.Muscle involvement was the most prominent and first manifestation,and the patient had widespread visceral involvements,which included cardiovascular system,kidney,respiratory as well as gastrointestinal tracts and tongue.The biopsy of the muscle,mucosa of stomach and intestine,and cutaneous tissue revealed amyloid material deposited in the skeletal and smooth muscle as well as vessel walls.Conclusion Amyloid myopathy is a rare manifestation in systemic amyloidosis.Skeletal muscle weakness and stiffening may be an important clue to the diagnosis of systemic amyloidosis.

Key words: Amyloidosis, Muscular diseases