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硬化性粘液水肿(全身性粘液水肿性苔藓)是一种罕见的纤维粘蛋白性结缔组织病,由Gottron于1954年最早报道,其临床特征为皮肤腊样丘疹伴受累皮肤的硬化,多数病人有血清单克隆丙球蛋白病。对本病的历史和系统性表现知之甚少。为了解本病的系统性表现,作者复习了1950～1985年间经活检证实为硬化性粘液水肿的19例患者的临床记录。其中10例男性,9例女性,确诊时年龄中位数为53岁,随访7个月～23年(中位数为4.5年)。皮肤以外的表现包括:13例有单克隆丙球蛋白病,8例主诉吞咽困难,3例近端食道机能障碍,1例吞钡检查时全食道蠕动停止,5例近端肌无力,3例有炎性肌病,6例主诉运动性呼吸困难,其中5
Sclerosing myxedema (generalized mucoid edematous lichen) is a rare fibronectin connective tissue disease, first reported by Gottron in 1954 and characterized clinically as papillary dermatitis with compromised skin sclerosis. Most patients have Serum monoclonal gammopathy. Little is known about the history and systematic performance of the disease. To understand the systematic manifestation of this disease, the authors reviewed the clinical records of 19 patients with sclerosing myxedema confirmed by biopsy between 1950 and 1985. Of these, 10 were males and 9 were females. The median age at diagnosis was 53 years and were followed up for 7 months to 23 years (median, 4.5 years). Other skin manifestations include: 13 cases of monoclonal gammopathies, 8 cases of dysphagia, 3 cases of proximal esophageal dysfunction, 1 case of barium ingestion, total esophageal motility stopped, 5 cases of proximal muscle weakness, 3 cases There are inflammatory myopathy, 6 patients complained of exercise-induced dyspnea, of which 5