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散发性迟发性皮肤卟啉症、皮肤结节病与HFE基因Cys282Tyr和His63Asp突变的复合杂合性——病例报告

Sporadic Porphyria Cutanea Tarda, Cutaneous Sarcoidosis, and Compound Heterozygosity of HFE Mutations Cys282Tyr and His63Asp-A Case Report.

作者信息

Kim Jowon L, Crawford Richard, Lano Ian Marie, Merkeley Hayley

机构信息

Department of Medicine University of British Columbia Vancouver Canada.

Department of Pathology University of British Columbia Vancouver Canada.

出版信息

EJHaem. 2025 May 27;6(3):e70054. doi: 10.1002/jha2.70054. eCollection 2025 Jun.

Abstract

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.

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)的遗传性或获得性缺陷引起的。通过血色素沉着症基因(HFE)突变改变铁稳态是与散发性PCT相关的众多易感因素之一。尽管结节病通常与PCT无关,但先前关于肝结节病的报道推测,肝肉芽肿通过直接干扰或免疫机制影响UROD活性。在此,我们描述了一例在(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背景下发生的获得性PCT病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a627/12109468/c6c2be1770d6/JHA2-6-e70054-g002.jpg

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