Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 46-year-old white man had classic findings of scleredema; subsequently, a monoclonal immunoglobulin G lambda light chain was detected in his serum. Findings of a bone marrow biopsy specimen revealed that he had an increased percentage of plasma cells, some of which were atypical. A diagnosis of scleredema and smoldering myeloma was made.
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PMID:Scleredema and smoldering myeloma. 156 50

Multiple myeloma and congestive heart failure developed in a patient with long-standing scleredema adultorum. Staining of the myocardium, performed after her death, was positive for acid mucopolysaccharide and negative for amyloid. To the best of our knowledge, this is the first case in which acid mucopolysaccharide has been demonstrated in the myocardium, thus explaining the cardiomyopathy of scleredema adultorum. Review of the world literature enabled us to identify a statistically significant increased prevalence of plasma cell dyscrasia among patients with protracted scleredema. In all patients, plasma cell dyscrasia appeared years after the onset of scleredema. Immunofluorescent studies were negative for immunoglobulin deposition. We assume, therefore, that the plasma cell dyscrasia was secondary to scleredema.
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PMID:Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum. 244 45

Scleredema associated with a monoclonal gammopathy and generalized skin pigmentation is described in a 56-year-old man with hyperlipoproteinemia and cardiovascular disease. The patient had IgG-lambda paraproteinemia, without any evidence of multiple myeloma or immunoglobulin deposition in affected skin. Ultrastructural studies of pigmented lesional skin showed increased transfer of melanosomes to basal keratinocytes and dermal melanophages containing complex melanosomes. In addition, cytoplasmic, electron-opaque lipid droplets were seen in approximately every third keratinocyte or melanocyte, while only an occasional dermal cell contained lipid droplets. The hyperpigmentation appeared to be directly related to the scleredema, while the lipid deposition in skin was a likely consequence of the hyperlipoproteinemia. The findings further support the contention that paraproteinemia and hyperpigmentation may, in some patients, be associated features of scleredema adultorum.
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PMID:Scleredema adultorum associated with a monoclonal gammopathy and generalized hyperpigmentation. 357 42

Two patients had scleredema with monoclonal gammopathy, one of whom was considered to have smoldering multiple myeloma. In one patient, the scleredema cleared without treatment, while in the other, the scleredema and the monoclonal gammopathy persisted unchanged.
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PMID:Scleredema and monoclonal gammopathy: report of two cases. 608 34

A monoclonal gammopathy was observed in 3 adult patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. The monoclonal immunoglobulin was in every case of the IgG-kappa type. Bence Jones proteinuria was noted in 1 case (kappa). Deposition of monoclonal IgG-kappa in the skin was not detected by immunofluorescence microscopy. These findings and previous reports of 5 cases suggest that diffuse scleredema may be frequently associated with paraproteinemia, but the role of monoclonal immunoglobulins in the pathogenesis of the disease remains to be clarified.
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PMID:[Buschke's scleredema and monoclonal dysglobulinemia: apropos of 3 cases]. 640 82

A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.
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PMID:Monoclonal gammopathy in scleredema. Observations in three cases. 679 60

Scleredema of Buschke is a rare disorder characterized by the development of areas of skin induration which usually resolve spontaneously. It is occasionally associated with a benign gammopathy, and rarely with myelomatosis. We describe a 60-year-old woman with extensive skin changes, who developed IgA myeloma. Unusually, her skin disease did not respond to conventional myeloma therapy. Death occurred as a consequence of the progressive skin disease.
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PMID:A fatal case of scleredema of Buschke. 820 80

The presence of a monoclonal gammopathy in patients with chronic scleredema is a recently described, unusual association of unknown significance. There have been very occasional reports of multiple myeloma in such patients. We now report a patient with long-standing scleredema who developed an IgA-kappa multiple myeloma. Once chemotherapy for the myeloproliferative disorder was instituted, scleredema improved significantly, suggesting a pathogenetic link between the two conditions.
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PMID:Improvement of scleredema associated with IgA multiple myeloma after chemotherapy. 856 52

We report a case of scleredema of Buschke associated with IgG kappa monoclonal hypergammaglobulinaemia. After myeloma polychemotherapy an improvement in skin involvement was observed and confirmed by means of noninvasive skin elasticity measurements. This suggests a relationship between the two diseases. The bioengineering method used can be useful for early detection and monitoring the skin involvement in patients with this disease association.
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PMID:Biomechanical measurement of skin distensibility in scleredema of Buschke associated with multiple myeloma. 1084 8

Scleredema adultorum, or Buschke's scleredema, belongs to the group of mucinoses. It is characterised by thickened and indurated skin. Histopathology shows thickened dermis with an infiltration of mucin between swollen collagen bundles. There are reports about many associations with scleredema adultorum, e.g., with diabetes mellitus and multiple myeloma. One case is known with associated primary hyperparathyroidism. For the first time we report a case of scleredema adultorum and secondary hyperparathyroidism, in a 46-year-old patient. Both forms of hyperparathyroidism have increased levels of parathormone. Therefore, these increased levels could have an influence on collagen metabolism.
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PMID:[Scleredema adultorum in secondary hyperparathyroidism]. 1196 92


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