Amiloidose localizada cutânea primária nodular: relato de caso

Abstract

Abstract:Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain. Keywords: Amyloidosis; Plasma cells; Skin Resumo: A amiloidose resulta da deposição de proteína amiloide fibrosa e insolúvel em espaços extracelulares de órgãos e tecidos. O depósito da substância amiloide pode ser localizado ou sistêmico e pode ser de natureza primária ou secundária. Relataremos um caso de amiloidose localizada cutânea primária nodular, manifesta por lesões pápulo-nodulares, eritêmato-acastanhadas, acometendo a região nasal, sem evidência de acometimento sistêmico. O estudo imunoistoquímico demonstrou presença de imunoglobulinas de cadeia leve kappa.

Nodular primary localized cutaneous amyloidosis: presentation of 2 case reports and literature review

“…6,8 The AL protein is not specific for the nodular cutaneous amyloidosis, it also exists in the primary systemic amyloidosis or the amyloidosis associated with multiple myeloma. There are reports of nodular cutaneous amyloidosis associated with systemic diseases like diabetes, 4 and autoimmune diseases, such as Sjögren's syndrome, 2,8,12 CREST syndrome, primary biliary cirrhosis, rheumatoid arthritis and systemic lupus erythematosus. 5,6,13 Other rare associations described in the literature include alcoholic cirrhosis, 11 atopic dermatitis 14,15 and sarcoidosis 7 .…”

“…Amyloidosis is a common term used to describe a collection of diseases that are characterized by extracellular deposition of insoluble fibrillar protein amyloid substance. 1,2,3,4,5 It can be divided into systemic and localized forms. 1,2,4,5 In the former, there is widespread deposition of amyloid substance in various organs and tissues, while in the latter the deposition of this material is restricted to a single organ or tissue.…”

“…1,2,3,4,5 It can be divided into systemic and localized forms. 1,2,4,5 In the former, there is widespread deposition of amyloid substance in various organs and tissues, while in the latter the deposition of this material is restricted to a single organ or tissue. 2 Both can be further subdivided into primary or secondary.…”

Acquired hyperpigmentations

“…The distinction between primary localized cutaneous amyloidosis and systemic forms must be made through a careful physical examination and laboratory tests to exclude the presence of extracutaneous amyloid deposits and plasmocytic dyscrasias. 105 …”

“…The process can have a localized benign chronic course, but patients should be monitored for progression to systemic amyloidosis and plasmocytic dyscrasias, which occurs in 7-50% of patients. 103,105,117 …”