Publication details
Scleroedema adultorum Buschke a scleromyxedema
| Title in English | Treatment of mucinoses - scleroedema adultorum Buschke and scleromyxedema with presence of monoclonal immunoglobulin |
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| Authors | |
| Year of publication | 2015 |
| Type | Article in Periodical |
| Magazine / Source | Česká dermatovenerologie |
| MU Faculty or unit | |
| 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. |