Craus Daniel, Kreuter Alexander, Bertling Frederik, Theodoropoulou Stela, Müller Valentina Laura
Department of Dermatology, Venereology and Allergology, Helios St. Johannes Klink Duisburg, Dieselstraße 185, 47166, Duisburg, Germany.
Department of Dermatology, Venereology and Allergology, Helios St. Elisabeth Klinik, Oberhausen, Germany.
Wien Med Wochenschr. 2025 Oct;175(13-14):309-310. doi: 10.1007/s10354-025-01095-7. Epub 2025 Jul 7.
This report emphasizes the importance of a thorough clinical examination for detecting bilateral periocular purpura, a key clinical hallmark of amyloid light-chain (AL) amyloidosis. Amyloid light-chain amyloidosis, often secondary to plasma cell dyscrasia, results from amyloid light-chain deposition, potentially leading to multiorgan involvement with progressive damage. Diagnosis relies on histopathology with Congo red staining, serum protein studies, and bone marrow analysis. Early recognition and treatment of the underlying hematologic malignancy are crucial for improving patient outcomes.
本报告强调了全面临床检查对于检测双侧眼周紫癜的重要性，双侧眼周紫癜是轻链型（AL）淀粉样变性的关键临床特征。轻链型淀粉样变性常继发于浆细胞异常增生，由轻链淀粉样蛋白沉积引起，可能导致多器官受累并进行性损害。诊断依赖刚果红染色的组织病理学检查、血清蛋白研究和骨髓分析。早期识别并治疗潜在的血液系统恶性肿瘤对于改善患者预后至关重要。
