[皮肤卟啉症晚期]

Q4 Medicine
Josephine Mistegård, Anne Lindegaard Christiansen, Christian Vestergaard, Hans Christian Wulf, Henrik F Lorentzen
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引用次数: 0

摘要

迟发性皮肤卟啉症(PCT)是由多种易感因素引起的尿卟啉原脱羧酶(UROD)活性下降引起的,这里是UROD的致病变异和铁超载的存在。卟啉对光敏感,在暴露在阳光下的皮肤上引起水泡和溃疡等皮肤症状。本文综述了PCT的诊断、监测和治疗的最新进展,PCT通常通过静脉切断术或/和低剂量羟氯喹有效治疗。此外,必须告知患者充分的防晒和减少易感因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Porphyria cutanea tarda].

Porphyria cutanea tarda (PCT) is caused by decreased activity of uroporphyrinogen decarboxylase (UROD) due to various susceptibility factors, here among pathogenic variants in UROD and the presence of iron overload. Porphyrins are sensitive to light and cause cutaneous symptoms in the form of blisters and ulcers on sun-exposed skin. This review gives an update on diagnosing, monitoring and the treatment of PCT. PCT is often effectively treated with venesection or/and low-dose hydroxychloroquine. Additionally, patients must be informed about sufficient sun protection and reduction of susceptibility factors.

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来源期刊
Ugeskrift for laeger
Ugeskrift for laeger Medicine-Medicine (all)
CiteScore
0.20
自引率
0.00%
发文量
345
期刊介绍: The Ulster Medical Journal is an international general medical journal with contributions on all areas of medical and surgical specialties relevant to a general medical readership. It retains a focus on material relevant to the health of the Northern Ireland population. The Honorary Editor would welcome offers of papers for publication. Prospective authors are invited to read the notice to contributors.
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