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着色性干皮病:光试验的作用

Xeroderma pigmentosum: the role of phototesting.

作者信息

Stone N, Reed J, Mahood J, Hardwick N, George S

机构信息

Photodermatology Unit, Amersham Hospital, Amersham, Buckinghamshire HP7 0JD, UK.

出版信息

Br J Dermatol. 2000 Sep;143(3):595-7. doi: 10.1111/j.1365-2133.2000.03716.x.

DOI:10.1111/j.1365-2133.2000.03716.x
PMID:10971335
Abstract

We report three patients newly diagnosed as having xeroderma pigmentosum (XP). Previous photobiological studies have implicated ultraviolet (UV) B as the activating waveband in XP, causing a delayed and prolonged erythemal response. This characteristic reaction pattern has been used as a quick screening test in patients suspected of having XP, while awaiting confirmatory DNA repair studies. Two of our patients showed no abnormal erythemal responses, and one showed severe photosensitivity from 330 to 400 nm but normal UVB responses, with a peak erythema at 24 h. We conclude that the erythemal responses in XP are highly variable and cannot be considered as a reliable screening test in the diagnosis of XP.

摘要

我们报告了三名新诊断为着色性干皮病(XP)的患者。先前的光生物学研究表明,紫外线(UV)B是XP中的激活波段,可导致延迟和延长的红斑反应。在等待确诊的DNA修复研究时,这种特征性反应模式已被用作疑似患有XP患者的快速筛查测试。我们的两名患者未表现出异常的红斑反应,一名患者在330至400nm处表现出严重的光敏性,但UVB反应正常,在24小时出现红斑峰值。我们得出结论,XP中的红斑反应高度可变,不能被视为诊断XP的可靠筛查测试。

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