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Although oral manifestations of amyloidosis are rare, it is important to exclude systemic involvement in patients who present with amyloidosis involving the oral cavity. Improved understanding of the oral manifestations of amyloidosis and the additional investigations required can lead to timely diagnosis and subsequent management. Treatment of localized forms of amyloidosis include resection, biologics, laser therapy and corticosteroids.
CPD/Clinical Relevance: Understanding the oral manifestations of amyloidosis can lead to timely diagnosis and management.
Article
Localized or systemic amyloidosis results from the abnormal folding of proteins, forming insoluble amyloid fibrils with subsequent deposition in various tissues and organs.1 Based upon the specific protein form that is deposited, it is classified as:
Localized amyloidosis occurs most commonly whereby the amyloid protein is restricted to a particular organ or tissue and results in deposition of AL (L stands for light chain, the protein that mutates and causes the disorder) protein.2 In systemic AL amyloidosis, previously termed primary amyloidosis, the AL deposits are located in any, or all, of the viscera, connective tissue and blood vessel walls. It is usually secondary to benign low-grade monoclonal gammopathy.3 Reactive systemic AA (serum amyloid A protein) amyloidosis, formerly known as secondary amyloidosis, occurs as a complication of chronic infections (for example tuberculosis) and inflammatory conditions (e.g. rheumatoid arthritis and Crohn's disease). It is characterized by an increased production of AA (serum amyloid A) protein.4 Hereditary systemic amyloidosis is rare and a result of autosomal dominant diseases in which there is production of amyloid fibrils from inherited variant proteins, such as transthyretin, gelsolin and apolipoprotein A-1 and fibrinogen.5
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