[Arndt-Gottron scleromyxedema and associated phenomena].
[Arndt-Gottron scleromyxedema and associated phenomena].
复制标题
[Arndt-Gottron 硬化粘液水肿及相关现象]。
DOI：
--
复制
发表时间：
1984
期刊：
影响因子：
--
通讯作者：
H. Schmidt
中科院分区：
文献类型：
--
作者：
E. Lang;M. Zabel;H. Schmidt
硬化性黏液水肿是一种少见的纤维黏液性结缔组织疾病，其特征为真皮内黏液物质的积聚。单克隆副蛋白是定期鉴定。文献综述（57例病例）显示硬化性粘液水肿与多发性骨髓瘤（8.7%）和Waldenström巨球蛋白血症（3.5%）异常相关。报告1例男性硬化性粘液水肿、IgG λ副蛋白血症和硬化性趾畸形（硬化性粘液水肿的特殊征象）。美法仑是严重病例的首选药物，但对硬指症无效。
Scleromyxedema is an uncommon fibromucinous connective tissue disease characterized by accumulation of mucinous material in the dermis. A monoclonal paraprotein is regularly identified. A review of the literature (57 cases) shows the exceptional association of scleromyxedema with multiple myeloma (8.7%) and macroglobulinemia Waldenström (3.5%). A man with scleromyxedema, IgG lambda paraproteinemia, and sclerodactylia--as a special sign of scleromyxedema--is reported. Melphalan is the drug of choice in serious cases, but not effective in sclerodactylia.