Mucocutane manifestaties van amyloïdose

- Amyloidosis is the collective term for a group of uncommon metabolic disorders in which insoluble amyloid protein-fibres are deposited in tissues and organs. Mucocutaneous manifestations are frequently found in this disease.

- The different types of amyloidosis are divided into a systemic and a non-systemic group. Systemic amyloidosis is characterised by amyloid deposits in several organs.

- In the most frequent type, amyloid light chain (AL) systemic amyloidosis, the skin is involved in 29-40 of the cases. These mucocutaneous manifestations are sometimes the first clue to the discovery of systemic involvement.

- The non-systemic group comprises primarily localised amyloid deposits in skin and mucosa.

- The treatment of localised mucocutaneous amyloidosis is aimed at the local changes themselves.

- The mucocutaneous manifestations due to systemic amyloidosis may improve when it is possible to treat the underlying disease successfully.

Ned Tijdschr Geneeskd. 2007;151:2032-8