布施克硬化性水肿:因子XIII治疗缓解。

C Venturi, E Zendri, M Santini, E Grignaffini, R Ricci, G De Panfilis
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摘要

我们描述的情况下,55岁的男子与躯干的布施克硬化剂水肿合并胰岛素依赖型糖尿病。由于(i)患者一般健康状况不佳,(ii)硬皮病与布施克硬皮病之间的相似性,以及(iii)众所周知的注射因子XIII对硬皮病的疗效,我们尝试用因子XIII静脉治疗。这种治疗导致运动明显增加,皮肤软化,同时超声检查和组织病理学改善。总之,据我们所知,这是第一例因子XIII成功用于治疗Buschke硬化性水肿的病例。
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Scleredema of Buschke: remission with factor XIII treatment.

We describe the case of a 55-year-old man with scleredema of Buschke of the torso complicated by insulin-dependent diabetes mellitus. Due to (i) the patient's poor general health status, (ii) the similarity between scleroderma and scleredema of Buschke, and (iii) the well known efficacy of factor XIII infusions in scleroderma, we attempted an intravenous treatment with factor XIII. This therapy resulted in marked increase of movements and in softening of the skin, together with ultrasonographic and histopathological improvements. In conclusion, to the best of our knowledge, this is the first case in which factor XIII has been successfully used for the treatment of scleredema of Buschke.

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