Three cases of localized cutaneous nodular amyloidosis in patients with limited systemic sclerosis and a brief literature review. Int J Womens Dermatol 2017 Jun;3(2):91-95
Date
06/01/2017Pubmed ID
28560302Pubmed Central ID
PMC5440445DOI
10.1016/j.ijwd.2016.11.003Scopus ID
2-s2.0-85009445650 (requires institutional sign-in at Scopus site) 7 CitationsAbstract
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.
