台湾一个家族中COL7A1基因的新型杂合子错义变异导致的自发性萎缩性表皮松解症。

IF 3.8 3区 医学 Q2 CELL BIOLOGY
Wound Repair and Regeneration Pub Date : 2024-07-01 Epub Date: 2024-02-28 DOI:10.1111/wrr.13159
Yi-Chia Tsai, Wei-Ting Tu, Chun-Lin Su, Yu-Wen Cheng, Pei-Ling Chi, Chao-Kai Hsu, Yang-Yi Chen
{"title":"台湾一个家族中COL7A1基因的新型杂合子错义变异导致的自发性萎缩性表皮松解症。","authors":"Yi-Chia Tsai, Wei-Ting Tu, Chun-Lin Su, Yu-Wen Cheng, Pei-Ling Chi, Chao-Kai Hsu, Yang-Yi Chen","doi":"10.1111/wrr.13159","DOIUrl":null,"url":null,"abstract":"<p><p>Self-improving dystrophic epidermolysis bullosa (DEB) is a genodermatosis that is inherited autosomal dominantly or recessively, and its clinical symptoms may improve or subside spontaneously. Herein, we report a case of self-improving DEB with COL7A1 p.Gly2025Asp variant. The diagnosis was made through histopathological, electron microscopic examination, and genetic testing. The same variant is also noted on his father, who presents with dystrophic toenails without any blisters. This study highlights that idiopathic nail dystrophy could be linked to congenital or hereditary disease. Furthermore, we conducted a review of the literature on the characteristics of reported cases of self-improving DEB with a personal or family history of nail dystrophy. The results supported our findings that nail dystrophy may be the sole manifestation in some family members. We suggest that individuals suffering from idiopathic nail dystrophy may seek genetic counselling when planning pregnancy to early evaluate the potential risk of hereditary diseases.</p>","PeriodicalId":23864,"journal":{"name":"Wound Repair and Regeneration","volume":" ","pages":"511-516"},"PeriodicalIF":3.8000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Self-improving dystrophic epidermolysis bullosa with a novel heterozygous missense variant in the COL7A1 gene in a Taiwanese family.\",\"authors\":\"Yi-Chia Tsai, Wei-Ting Tu, Chun-Lin Su, Yu-Wen Cheng, Pei-Ling Chi, Chao-Kai Hsu, Yang-Yi Chen\",\"doi\":\"10.1111/wrr.13159\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Self-improving dystrophic epidermolysis bullosa (DEB) is a genodermatosis that is inherited autosomal dominantly or recessively, and its clinical symptoms may improve or subside spontaneously. Herein, we report a case of self-improving DEB with COL7A1 p.Gly2025Asp variant. The diagnosis was made through histopathological, electron microscopic examination, and genetic testing. The same variant is also noted on his father, who presents with dystrophic toenails without any blisters. This study highlights that idiopathic nail dystrophy could be linked to congenital or hereditary disease. Furthermore, we conducted a review of the literature on the characteristics of reported cases of self-improving DEB with a personal or family history of nail dystrophy. The results supported our findings that nail dystrophy may be the sole manifestation in some family members. We suggest that individuals suffering from idiopathic nail dystrophy may seek genetic counselling when planning pregnancy to early evaluate the potential risk of hereditary diseases.</p>\",\"PeriodicalId\":23864,\"journal\":{\"name\":\"Wound Repair and Regeneration\",\"volume\":\" \",\"pages\":\"511-516\"},\"PeriodicalIF\":3.8000,\"publicationDate\":\"2024-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Wound Repair and Regeneration\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1111/wrr.13159\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/2/28 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q2\",\"JCRName\":\"CELL BIOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Wound Repair and Regeneration","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/wrr.13159","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/2/28 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"CELL BIOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

自发性萎缩性表皮松解症(DEB)是一种常染色体显性或隐性遗传的遗传性皮肤病,其临床症状可自行改善或消退。在此,我们报告了一例伴有 COL7A1 p.Gly2025Asp 变异的自我改善型 DEB 病例。该病例通过组织病理学、电子显微镜检查和基因检测确诊。他的父亲也出现了同样的变异,表现为萎缩性脚趾甲,但没有任何水疱。这项研究强调,特发性甲营养不良症可能与先天性或遗传性疾病有关。此外,我们还对文献进行了回顾,以了解有指甲营养不良症个人史或家族史的自发性甲营养不良症病例的特征。结果支持了我们的发现,即指甲营养不良可能是某些家族成员的唯一表现。我们建议特发性甲营养不良症患者在计划怀孕时可寻求遗传咨询,以尽早评估遗传疾病的潜在风险。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Self-improving dystrophic epidermolysis bullosa with a novel heterozygous missense variant in the COL7A1 gene in a Taiwanese family.

Self-improving dystrophic epidermolysis bullosa (DEB) is a genodermatosis that is inherited autosomal dominantly or recessively, and its clinical symptoms may improve or subside spontaneously. Herein, we report a case of self-improving DEB with COL7A1 p.Gly2025Asp variant. The diagnosis was made through histopathological, electron microscopic examination, and genetic testing. The same variant is also noted on his father, who presents with dystrophic toenails without any blisters. This study highlights that idiopathic nail dystrophy could be linked to congenital or hereditary disease. Furthermore, we conducted a review of the literature on the characteristics of reported cases of self-improving DEB with a personal or family history of nail dystrophy. The results supported our findings that nail dystrophy may be the sole manifestation in some family members. We suggest that individuals suffering from idiopathic nail dystrophy may seek genetic counselling when planning pregnancy to early evaluate the potential risk of hereditary diseases.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Wound Repair and Regeneration
Wound Repair and Regeneration 医学-皮肤病学
CiteScore
5.90
自引率
3.40%
发文量
71
审稿时长
6-12 weeks
期刊介绍: Wound Repair and Regeneration provides extensive international coverage of cellular and molecular biology, connective tissue, and biological mediator studies in the field of tissue repair and regeneration and serves a diverse audience of surgeons, plastic surgeons, dermatologists, biochemists, cell biologists, and others. Wound Repair and Regeneration is the official journal of The Wound Healing Society, The European Tissue Repair Society, The Japanese Society for Wound Healing, and The Australian Wound Management Association.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信