每日一题_2024.08.10

A 58-year-old man presented to the dermatology clinic with a 2-year history of eyelid lesions and with weight loss and fatigue over the past several months. On physical examination, scattered periorbital petechiae and purpura were noted, as well as coalescing, waxy papules on the eyelids. Macroglossia was also present. Serum coagulation tests were normal. A biopsy of the left upper eyelid resulted in ecchymosis. What is the most likely explanation for the spontaneous periorbital petechiae and significant bruising after the biopsy in this case?

一位58岁男性患者因2年的眼睑病变史以及过去几个月体重下降和疲劳症状到皮肤科诊所就诊。在体格检查中，发现散在的眼眶周围瘀点和紫癜，以及眼睑上融合的蜡样丘疹。还观察到巨舌症。血清凝血试验正常。左上眼睑活检后出现瘀斑。在这种情况下，自发性眼眶周围瘀点和活检后显著瘀伤最可能的解释是什么？

A. Aspirin use阿司匹林使用

B. Chronic alcohol use长期饮酒

C. Factor X deficiency第十因子缺乏

D. Vascular fragility owing to infiltration of vessel walls因血管壁浸润导致的血管脆弱性

E. Vitamin C deficiency维生素C缺乏

答案解析：

根据活检标本的组织病理学检测和直接免疫荧光检测，结合血清游离轻链检测、尿蛋白电泳和骨髓活检的结果，诊断为AL型淀粉样变性。在AL型淀粉样变性（也称为淀粉样轻链或原发性淀粉样变性）中，淀粉样物质浸润血管壁导致血管脆弱性。可能出现容易瘀伤的情况——包括眼眶周围“浣熊眼”模式——并可能在受到外伤后加重，正如这位患者在活检后所发生的那样。

正确答案：D