散发性迟发性皮肤卟啉症、皮肤结节病及HFE突变Cys282Tyr和his63asp的复合杂合性1例报告

EJHaem Pub Date : 2025-05-27 DOI:10.1002/jha2.70054
Jowon L. Kim, Richard Crawford, Ian Marie Lano, Hayley Merkeley
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引用次数: 0

摘要

迟发性皮肤卟啉症(PCT)是由血红素生物合成途径中尿卟啉原脱羧酶(UROD)的遗传或获得性缺陷引起的。血色素沉着病基因(HFE)突变导致的铁稳态改变是与散发性PCT相关的众多易感因素之一。尽管结节病通常与PCT无关,但先前的肝结节病报道认为肝肉芽肿通过直接干扰或免疫机制影响UROD活性。在此,我们描述一例获得性PCT的情况下(HFE):c.845G> a (p.Cys282Tyr)和(HFE):c.187C>G (p.His63Asp)复合杂合性,肝脂肪变性和皮肤结节病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sporadic Porphyria Cutanea Tarda, Cutaneous Sarcoidosis, and Compound Heterozygosity of HFE Mutations Cys282Tyr and His63Asp—A Case Report

Porphyria cutanea tarda (PCT) is caused by inherited or acquired defects of uroporphyrinogen decarboxylase (UROD) in the heme biosynthetic pathway. Altered iron homeostasis via hemochromatosis gene (HFE) mutations is one of many susceptibility factors associated with the sporadic form of PCT. Though sarcoidosis is not commonly associated with PCT, prior reports of hepatic sarcoidosis postulated that hepatic granulomas affect UROD activity by direct interference or immunological mechanisms. Here we describe a case of acquired PCT in the setting of (HFE):c.845G>A (p.Cys282Tyr) and (HFE):c.187C>G (p.His63Asp) compound heterozygosity, hepatic steatosis, and cutaneous sarcoidosis.

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