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[阿恩特-戈特龙硬皮黏液水肿的内科疾病表现]

[Internal disease findings in Arndt-Gottron scleromyxedema].

作者信息

Lang E, Goos M

出版信息

Dtsch Med Wochenschr. 1986 May 23;111(21):820-3. doi: 10.1055/s-2008-1068538.

Abstract

A case of papular mucinosis (scleromyxoedema of Arndt-Gottron) in a 64-year-old man is reported. Although a cutaneous mucinosis, the disease is characterized by a number of systemic disorders which determine the course and prognosis of the disease. A survey of 57 reported cases reveals that, in addition to the obligatory paraproteinaemia, neurological (24% of cases), cardiovascular (10%) and myopathic (9%) symptoms are in the foreground. Systemic lymphoplasmo-reticular involvement occurred in 14%. These findings emphasize the need for interdisciplinary cooperation in the diagnosis and for long-term supervision of patients with this disease.

摘要

报告了一例64岁男性的丘疹性黏蛋白病(阿恩特-戈特龙硬皮黏液水肿)。尽管这是一种皮肤黏蛋白病,但该疾病的特征是存在许多决定疾病进程和预后的全身性疾病。对57例报告病例的调查显示,除了必然存在的副蛋白血症外,神经系统症状(24%的病例)、心血管系统症状(10%)和肌病症状(9%)最为突出。14%的病例出现全身性淋巴浆细胞网状组织受累。这些发现强调了在诊断中需要跨学科合作以及对该疾病患者进行长期监测。

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