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伴有罕见全身大疱和脆甲附加特征的 NAXOS-Carvajal 综合征变异型:病例报告及文献复习。

Variant NAXOS-Carvajal Syndrome with Rare Additional Features of Systemic Bulla and Brittle Nails: A Case Report and Literature Review.

机构信息

Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2021 Apr 1;60(7):1119-1126. doi: 10.2169/internalmedicine.5899-20. Epub 2020 Nov 2.

Abstract

Skin abnormalities are often indicative of cardiovascular diseases. Such a disease entity is called cardiocutaneous syndrome; however, the details regarding the involvement of bulla and nails remain largely unclear. A 49-year-old man with systemic bulla was admitted for heart failure. His bulla had previously been diagnosed as epidermolysis bullosa, but no known gene mutations for it had been identified. He had a triad of palmoplantar keratosis, curly and fine hair, and cardiomyopathy, which are characteristic of NAXOS-Carvajal syndrome. This case highlights the fact that bulla and brittle nails can accompany NAXOS-Carvajal syndrome, showing that these extra-cardiac findings can help identify otherwise overlooked serious cardiac conditions.

摘要

皮肤异常常提示心血管疾病。有一种疾病实体称为心皮综合征;然而,关于水疱和指甲的具体参与情况仍不清楚。一名 49 岁男性因心力衰竭入院,全身有大疱。他的水疱先前被诊断为大疱性表皮松解症,但没有发现已知的基因突变。他患有三联征:掌跖角化过度、卷发和细软毛发以及心肌病,这是 NAXOS-Carvajal 综合征的特征。该病例强调了水疱和脆弱指甲可伴随 NAXOS-Carvajal 综合征发生的事实,表明这些心脏外表现有助于识别否则可能被忽视的严重心脏疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8746/8079904/61a4f1ffdc98/1349-7235-60-1119-g001.jpg

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引用本文的文献

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