What is the morbidity associated with nodular localized cutaneous amyloidosis (NLCA)?

Updated: Nov 15, 2016
  • Author: Nicholas V Nguyen, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Answer

Nodular localized cutaneous amyloidosis typically is benign and limited to the skin. However, lesions are more often persistent. Reported rates of progression to systemic disease are derived from case series with small numbers of patients; these rates vary from 7% to nearly 50%. [3, 4] Progression to fatal systemic amyloidosis has been reported, although this is a rare occurrence. [5] As many as 25% of reported cases have been associated with Sjögren syndrome. Some speculate that these 2 disorders have may have a shared pathogenesis. [6, 7, 8, 9, 10]

Case reports have also correlated nodular localized cutaneous amyloidosis with other autoimmune disorders such as CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome), primary biliary cirrhosis, rheumatoid arthritis, and systemic lupus erythematosus. [11] Other rare associations described in the case report literature include alcoholic cirrhosis [12] and severe atopic dermatitis. [13]


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!