Ma Han, Chen Meilan, Li Juan, Li Ying, Qiu Shu
Third Affiliated Hospital, Sun Yat-sen University, Guangdong, China.
First Affiliated Hospital, Sun Yat-sen University, Guangdong, China.
An Bras Dermatol. 2015 Mar-Apr;90(2):270-1. doi: 10.1590/abd1806-4841.20153320.
Immunoglobulin light chain amyloidosis is the most common acquired systemic amyloidosis. Its presentation is often insidious and progressive, which may delay diagnosis. The authors describe a rare case of immunoglobulin light chain amyloidosis in a 34-year-old man with scleroderma-like manifestation substantiated by multifarious laboratory investigations and the histopathologic feature of involved skin lesions stained with Congo red and crystal violet. This helps to maintain a high clinical suspicion of the disease when confronting similar skin presentation.
免疫球蛋白轻链淀粉样变性是最常见的后天性全身性淀粉样变性。其表现往往隐匿且呈进行性,这可能会延迟诊断。作者描述了一例罕见的免疫球蛋白轻链淀粉样变性病例,患者为一名34岁男性,具有硬皮病样表现,通过多种实验室检查以及刚果红和结晶紫染色的受累皮肤病变的组织病理学特征得以证实。这有助于在面对类似皮肤表现时对该疾病保持高度的临床怀疑。