埃洛珀持续性肢端皮炎和全身性脓疱性银屑病:兄弟姐妹中IL36RN突变两种不同表现的病例报告。

IF 5.2 Q1 DERMATOLOGY
Psoriasis (Auckland, N.Z.) Pub Date : 2025-03-26 eCollection Date: 2025-01-01 DOI:10.2147/PTT.S498720
Mengjiao Gu, Hanjing Huang, Zhanshuo Xiao, Fanzhang Meng, Han Sheng, Zhimin Lin, Chen Li, Yuanhao Wu
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引用次数: 0

摘要

在我们的论文中,我们提出了一个病例研究,兄弟姐妹患有广泛性脓疱性银屑病(GPP)和埃洛珀持续肢端皮炎(ACH),两者都携带IL36RN基因突变。3岁先证者表现出全身性脓疱,导致GPP诊断,而他6岁的妹妹出现了ACH特征的指甲溃疡和趾下脓疱。尽管进行了标准治疗,但他们的病情是难治性的。遗传分析显示为纯合剪接变异体C .115+6 T>C,亲本为杂合。本病例强调了IL36RN突变在脓疱性银屑病中的作用,并支持ACH作为该疾病的局部形式。具有相同突变的兄弟姐妹中不同的亚型表明受其他因素影响的复杂发病机制。我们的研究结果强调了基因检测在脓疱性银屑病中的重要性,并为进一步研究il36rn相关疾病的表型变异性提供了依据。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Acrodermatitis Continua of Hallopeau and Generalised Pustular Psoriasis: Case Reports of Two Different Manifestations of IL36RN Mutation in Siblings.

Acrodermatitis Continua of Hallopeau and Generalised Pustular Psoriasis: Case Reports of Two Different Manifestations of IL36RN Mutation in Siblings.

Acrodermatitis Continua of Hallopeau and Generalised Pustular Psoriasis: Case Reports of Two Different Manifestations of IL36RN Mutation in Siblings.

Acrodermatitis Continua of Hallopeau and Generalised Pustular Psoriasis: Case Reports of Two Different Manifestations of IL36RN Mutation in Siblings.

In our manuscript, we present a case study of siblings with Generalized Pustular Psoriasis (GPP) and Acrodermatitis Continua of Hallopeau (ACH), both harboring IL36RN gene mutations. The 3-year-old proband exhibited systemic pustules leading to a GPP diagnosis, while his 6-year-old sister developed nail ulcers and subungual pustules characteristic of ACH. Despite standard treatments, their conditions were refractory. Genetic analysis revealed a homozygous splice variant c.115+6 T>C, with heterozygous parents. This case underscores the role of IL36RN mutations in pustular psoriasis and supports ACH as a localized form of the disease. The distinct subtypes in siblings with identical mutations suggest a complex pathogenesis influenced by additional factors. Our findings highlight the importance of genetic testing in pustular psoriasis and warrant further investigation into the phenotypic variability of IL36RN-related disease.

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