[Treatment of mucinoses - scleroedema adultorum Buschke and scleromyxedema with presence of monoclonal immunoglobulin]

Details

Serval ID
serval:BIB_72E9D3FB20E5
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Title
[Treatment of mucinoses - scleroedema adultorum Buschke and scleromyxedema with presence of monoclonal immunoglobulin]
Journal
Ceska dermatovenerologie
Author(s)
Adam Z., Litzman J., Szturz Petr, Krejci M., Vasku V., Pour L., Michalkova E., Sevcikova S., Cermakova Z., Vesely K., Vanicek J., Pourova E., Kral Z.
Publication state
Published
Issued date
2015
Peer-reviewed
Oui
Volume
5
Number
3
Pages
150-158
Language
Czech
Abstract
Mucinoses of the scleroedema or scleromyxedema type are diseases characteristic by excessive formation of mucin deposits in the skin and hypodermis, which makes the skin inflexible. The deposits limit the movement of the limbs, the chest but also the mouth. Internal organs can be damaged by the same mechanism (heart, lungs, digestive tract). The cause of these changes is probably stimulation of mucin formation in the fibroblasts by immunoglobulins, usually monoclonal immunoglobulin. This is why these diseases are often associated with monoclonal gamapathy. To treat mucinoses associated with monoclonal gamapathy, chemotherapy can be used, specifically high-dose chemotherapy used for multiple myeloma. If this treatment is impossible or remains inefficient, immunoglobulins can be applied intravenously. The latter treatment removes the dermal symptoms, but does not lead to lowering the level of monoclonal immunoglobulin.
Create date
06/01/2025 19:46
Last modification date
07/01/2025 7:04
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