Publication details

Scleroedema adultorum Buschke a scleromyxedema

Title in English Treatment of mucinoses - scleroedema adultorum Buschke and scleromyxedema with presence of monoclonal immunoglobulin
Authors

ADAM Zdeněk LITZMAN Jiří SZTURZ Petr KREJČÍ Marta VAŠKŮ Vladimír POUR Luděk ŠEVČÍKOVÁ Eva ŠEVČÍKOVÁ Sabina ČERMÁKOVÁ Z. VESELÝ Karel VANÍČEK Jiří POUROVÁ E. KRÁL Zdeněk

Year of publication 2015
Type Article in Periodical
Magazine / Source Česká dermatovenerologie
MU Faculty or unit

Faculty of Medicine

Citation
Field Oncology and hematology
Keywords mucinosis; scleroedema; scleromyxedema; papular mucinosis; monoclonal gamapathy of uncertain significance; multiple myeloma; monoclonal immunoglobulin
Description 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, diges-tive 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.

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