[Mucocutaneous manifestations of amyloidosis].
Amyloidosis is the collective term for a group ofuncommon 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 ofamyloidosis 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.
Timmer-de Mik
L
L
Universitair Medisch Centrum Utrecht, Postbus 85500, 3508 GA Utrecht. l.timmer@umcutrecht.nl
van der Waal
R I F
RI
Canninga-van Dijk
M R
MR
Dikland
W J
WJ
Rodenburg
C J
CJ
Bruijnzeel-Koomen
C A F M
CA
D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000175:diagnosis / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D012867:Skin / Q000473:pathology*
D000682:Amyloid