Goettsche L S, Moye M S, Tschetter A J, Stone M S, Wanat K A
University of Iowa Carver College of Medicine, Iowa City, IA.
Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, IA.
Int J Womens Dermatol. 2017 Jan 11;3(2):91-95. doi: 10.1016/j.ijwd.2016.11.003. eCollection 2017 Jun.
Localized cutaneous nodular amyloidosis (LCNA) is the rarest form of localized cutaneous amyloidosis. In patients with LCNA, local plasma cells secrete immunoglobulin light chains called amyloid L via an unknown mechanism. LCNA has been associated with autoimmune connective tissue diseases such as most commonly Sjögren syndrome. A few reported cases of LCNA are associated with limited systemic sclerosis (LSSc). We report three cases of LCNA in patients with LSSc to add to the existing literature, discuss the disease association and proposed pathophysiology, and briefly review the existing information in current literature. It is important to closely follow patients with LCNA to monitor progression to systemic amyloidosis.
局限性皮肤结节性淀粉样变性（LCNA）是局限性皮肤淀粉样变性最罕见的形式。在LCNA患者中，局部浆细胞通过未知机制分泌称为淀粉样蛋白L的免疫球蛋白轻链。LCNA与自身免疫性结缔组织疾病相关，最常见的是干燥综合征。少数报道的LCNA病例与局限性系统性硬化症（LSSc）有关。我们报告3例LSSc患者的LCNA病例以补充现有文献，讨论疾病关联和提出的病理生理学，并简要回顾当前文献中的现有信息。密切随访LCNA患者以监测其进展为系统性淀粉样变性非常重要。
