先天性白细胞黏附糖蛋白缺乏症（CDLG）的皮肤表现
Skin manifestations in congenital deficiency of leucocyte-adherence glycoproteins (CDLG).
作者信息
van de Kerkhof P C, Weemaes C M
机构信息
Department of Dermatology, University Hospital of Nijmegen, The Netherlands.
出版信息
Br J Dermatol. 1990 Sep;123(3):395-401. doi: 10.1111/j.1365-2133.1990.tb06301.x.
Abstract
In congenital deficiency of leucocyte-adherence glycoproteins (CDLG) there is an immunodeficiency with impaired leucocyte function and cutaneous and extracutaneous infections occur. In more than 30% of cases the condition has a fatal course. We report the skin manifestations of three siblings with CDLG in which areas of skin necrosis occurred that resembled pyoderma gangrenosum.
摘要
在先天性白细胞黏附糖蛋白（CDLG）缺乏症中，存在免疫缺陷，白细胞功能受损，会发生皮肤和皮肤外感染。超过30%的病例病情呈致命性发展。我们报告了三名患有CDLG的兄弟姐妹的皮肤表现，他们出现了类似坏疽性脓皮病的皮肤坏死区域。
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