获得性皮肤松弛症合并肺气肿1例并文献复习
孙雪峰;钟旭;
摘要(Abstract)：
目的研究皮肤松弛症合并肺气肿的临床表现、病理特点及治疗。方法报告1例获得性皮肤松弛症合并肺气肿患者的诊治及文献复习。结果 1例26岁男性患者2年余前腹部、双胁、双腹股沟区多处出现皮炎,皮疹处皮肤逐渐松弛;1年余前开始出现活动后气短症状,进行性加重。肺功能检查提示极严重阻塞性通气功能障碍,胸部高分辨CT检查提示双侧全小叶型肺气肿。皮肤活检证实为皮肤松弛症,胸腔镜下肺活检见肺气肿及多个肺大泡形成。肺组织电镜提示肺泡间隔增宽,间隔内未见成结构的弹性纤维,可见大量胶原纤维增生。予以持续小流量吸氧,并予沙美特罗替卡松、噻托溴铵吸入治疗,病情维持稳定,复查肺功能略有好转。结论获得性皮肤松弛症合并肺气肿极其罕见,弹性纤维破坏是其共同特征,但其病原学至今仍不清楚。此类患者预后不佳,家庭氧疗、支气管扩张剂和吸入激素治疗可能有利于改善其肺功能。
关键词(KeyWords)：
皮肤松弛症;肺气肿;弹性纤维
基金项目(Foundation):
作者(Author):
孙雪峰;钟旭;
Email:
DOI:
参考文献(References)：
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