Department of Dermatology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
Chin Med J (Engl). 2012 Feb;125(3):555-7.
Primary systemic amyloidosis is a relatively rare disease, caused when abnormal extracellular deposition of fibrillary protein builds up in a variety of target organs, such as heart, kidneys, lungs liver, and so forth. The symptoms of the disease are usually vague, while many kinds of auxiliary or laboratory examinations especially pathologic biopsy can provide a clue for the diagnosis. Here we described a case who had purpura-like lesions in the initial stage, followed by progressive malfunctions in the kidneys, the heart, the lungs, as well as the liver. The final diagnosis was primary systemic amyloidosis determined by skin pathologic biopsy. And the disease led to a fatal outcome within three months after the diagnosis.
原发性系统性淀粉样变是一种相对罕见的疾病,当异常的细胞外纤维状蛋白在多种靶器官(如心脏、肾脏、肺、肝脏等)中积累时就会发生。该疾病的症状通常较为模糊,而多种辅助或实验室检查,尤其是病理活检可以为诊断提供线索。我们在此描述了 1 例以初期紫癜样皮损为特征,随后出现进行性肾脏、心脏、肺和肝脏功能障碍的患者。最终通过皮肤病理活检确定诊断为原发性系统性淀粉样变。该疾病在诊断后 3 个月内导致了致命后果。