Cases reported "Hypergammaglobulinemia"

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1/4. Scleredema and smoldering myeloma.

    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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ranking = 1
keywords = scleredema
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2/4. scleredema adultorum of Buschke: a case report and review of the literature.

    OBJECTIVES: To present a case of scleredema adultorum of Buschke associated with hypergammaglobulinemia and review the literature pertaining to this disease. methods: Search of medline (pubmed) was performed using the words "scleredema adultorum." Only cases in the English language, with pertinent clinical information for analysis, were included. RESULTS: We present a case of scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with scleredema. Our patient was found to have a B-cell lymphoma before being diagnosed with scleredema. The progression of skin thickening halted with no apparent correlation to immunosuppressive therapies or chemotherapeutic agents. A total of 165 cases of scleredema adultorum are described in the literature. There are 3 types of scleredema adultorum. Type 1 is usually preceded by a febrile episode and resolves spontaneously. Type 2 is associated with developing paraproteinemias including multiple myeloma. Type 3 is associated with diabetes mellitus. CONCLUSIONS: scleredema adultorum of Buschke is a rare disorder which is sometimes associated with hypergammaglobulinemia; our patient had a B-cell lymphoma. physicians should suspect scleredema in any patient with diffuse skin thickening where the hands and feet are spared, particularly if diabetes or a preceding febrile episode were present. Once the diagnosis of scleredema is made, evidence for the presence of hypergammaglobulinemia should be sought.
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ranking = 42.808667691246
keywords = scleredema adultorum, adultorum, scleredema
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3/4. Scleredema and monoclonal gammopathy: report of two cases.

    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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ranking = 1.5
keywords = scleredema
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4/4. Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia.

    We report a case of scleredema adultorum (Buschke's disease) associated with an IgA kappa monoclonal hypergammaglobulinaemia. A significant improvement in the skin was obtained with electron-beam therapy. Scleredema would appear to be linked to monoclonal hypergammaglobulinaemia, but the relationship between the skin disorder and the immunoglobulin abnormality remains to be elucidated.
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ranking = 53.005878783033
keywords = scleredema adultorum, adultorum, scleredema
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