Primary localized cutaneous nodular amyloidosis associated with CREST (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome.

摘要

Primary localized cutaneous amyloidosis (PLCA) is a rare condition that presents with the deposition of amyloid material in the skin, with no evidence of systemic involvement. Primary localized cutaneous nodular amyloidosis (PLCNA) is the rarest type of cutaneous amyloidosis. Of the three subdivisions of localized amyloidosis, macular and lichenoid subtypes show keratin-derived amyloid, whereas the nodular subtype is believed to be derived from plasma cells. Several disorders, including systemic sclerosis, primary biliary cirrhosis, systemic lupus erythematosus, Sjogren's syndrome, and rheumatoid arthritis, have been associated with PLCA. Summers and Kendrick recently presented the first case of PLCNA with the CREST variant (calcinosis, Raynaud's phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) of systemic sclerosis. To our knowledge, we report the second case of this association.