Dermatological Manifestations

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bookPart
Data de publicação
2023
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SPRINGER INTERNATIONAL PUBLISHING
Citação
Cury-Martins, J.; Sanches, J. A.. Dermatological Manifestations. In: . Amyloidosis and Fabry Disease: a Clinical Guide: SPRINGER INTERNATIONAL PUBLISHING, 2023. p.97-105.
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Resumo
Cutaneous involvement by amyloidosis is characterized by extracellular deposition of amyloid protein within the skin and appendages and can occur as a skin-limited disorder (localized cutaneous amyloidosis—LCA) or as a manifestation of systemic amyloidosis (SA). LCA can be divided into primary LCA—macular, lichen and nodular amyloidosis; or secondary LCA. The main subtypes of SA with cutaneous involvement include immunoglobulin light chain (AL) amyloidosis, secondary (AA) amyloidosis, dialysis-related amyloidosis, and heredofamilial amyloidosis. Skin involvement is present, for example, in approximately 40% of patients with AL amyloidosis, and might provide an early clue to the existence of an underlying plasma-cell dyscrasia. © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2023.
Palavras-chave
Lichen amyloidosis, Localized cutaneous amyloidosis, Macular amyloidosis, Nodular amyloidosis, Skin, Systemic amyloidosis with cutaneous involvement
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