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迟发性皮肤卟啉病

Porphyria cutanea tarda.

作者信息

Khoo B P, Tay Y K

机构信息

Raffles Surgicentre, Singapore.

出版信息

Singapore Med J. 2000 Jun;41(6):292-4.

Abstract

Porphyrias are a group of rare metabolic disorders in which excessive quantities of porphyrins, or their precursors, are produced. They are due to specific enzyme deficiencies resulting in abnormalities in the control of the porphyrin-haem metabolic pathway. Porphyria cutanea tarda (PCT) is the most common of all the porphyrias. However this condition is rarely seen in our Asian countries. We describe a patient with PCT who presented clinically with blistering eruptions over the sun-exposed areas. Coral pink fluorescence of uroporphyrins in an acidified urine specimen is diagnostic. Definitive treatment involves the use of low-dose chloroquine and interval venesection.

摘要

卟啉病是一组罕见的代谢紊乱疾病,其中会产生过量的卟啉或其前体。它们是由特定的酶缺乏导致卟啉 - 血红素代谢途径控制异常引起的。迟发性皮肤卟啉病(PCT)是所有卟啉病中最常见的。然而,这种病症在我们亚洲国家很少见。我们描述了一名患有PCT的患者,其临床表现为暴露于阳光下的部位出现水疱性皮疹。酸化尿液标本中尿卟啉的珊瑚粉红色荧光具有诊断意义。明确的治疗包括使用低剂量氯喹和间歇性静脉放血。

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