鱼鳞病。常染色体显性遗传型与性连锁型鱼鳞病临床体征鉴别的可靠性。

Ichthyosis. Reliability of clinical signs in the differentiation between autosomal dominant and sex-linked forms.

作者信息

Bousema M, van Diggelen O, van Joost T, Stolz E, Naafs B

机构信息

Department of Dermato-Venereology, University Hospital Rotterdam-Dijkzigt, The Netherlands.

出版信息

Int J Dermatol. 1989 May;28(4):240-2. doi: 10.1111/j.1365-4362.1989.tb04812.x.

DOI:10.1111/j.1365-4362.1989.tb04812.x

Abstract

The reliability of clinical signs to differentiate between autosomal dominant ichthyosis (ADI) or sex-linked ichthyosis (SLI) were assessed. SLI was defined as ichthyosis in combination with steroid sulphatase deficiency. Age of onset and format of the scales were found to be reliable signs to ascertain the diagnosis of ADI or SLI. The ADI-like appearance in histopathology seems to be of little diagnostic value.

摘要

评估了临床体征鉴别常染色体显性鱼鳞病（ADI）或性联鱼鳞病（SLI）的可靠性。SLI被定义为鱼鳞病合并类固醇硫酸酯酶缺乏症。发现发病年龄和鳞屑形态是确诊ADI或SLI的可靠体征。组织病理学中类似ADI的表现似乎诊断价值不大。

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