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Current approaches to the diagnosis and treatment of white sponge nevus

Published online by Cambridge University Press:  29 May 2015

Wenping Cai
Affiliation:
Department of Stomatology, Huashan Hospital, Fudan University, Shanghai 200040, People's Republic of China
Beizhan Jiang
Affiliation:
Laboratory of Oral Biomedical Science and Translational Medicine, School of Stomatology, Tongji University, Shanghai 200072, People's Republic of China
Fang Yu
Affiliation:
Laboratory of Oral Biomedical Science and Translational Medicine, School of Stomatology, Tongji University, Shanghai 200072, People's Republic of China
Jianhua Yang
Affiliation:
Department of Ophthalmology, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, People's Republic of China
Zhenghu Chen
Affiliation:
Department of Ophthalmology, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, People's Republic of China
Junjun Liu
Affiliation:
Department of Ophthalmology, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, People's Republic of China
Rongbin Wei
Affiliation:
Department of Ophthalmology, Shanghai Tenth People's Hospital, Tongji University School of Medicine, Shanghai 200072, People's Republic of China
Shouliang Zhao*
Affiliation:
Department of Stomatology, Huashan Hospital, Fudan University, Shanghai 200040, People's Republic of China
Xiaoping Wang*
Affiliation:
Laboratory of Oral Biomedical Science and Translational Medicine, School of Stomatology, Tongji University, Shanghai 200072, People's Republic of China
Shangfeng Liu*
Affiliation:
Department of Stomatology, Huashan Hospital, Fudan University, Shanghai 200040, People's Republic of China
*
*Shouliang Zhao, Department of Stomatology, Huashan Hospital, Fudan University, Shanghai 200040, People's Republic of China Tel: 86-21-52887813; Fax: 86-21-52887813; E-mail: slzhao@tongji.edu.cn
*Xiaoping Wang, Laboratory of Oral Biomedical Science and Translational Medicine, School of Stomatology, Tongji University, Shanghai 200072, People's Republic of China Tel: 86-21-65986073; Fax: 86-21-65986073; E-mail: wxp990@hotmail.com
*Corresponding author: Shangfeng Liu, Department of Stomatology, Huashan Hospital, Fudan University, Shanghai 200040, People's Republic of China Tel: 86-21-65986790; Fax: 86-21-65986790; E-mail: shangfengliufudan@163.com

Abstract

White sponge nevus (WSN) in the oral mucosa is a rare autosomal dominant genetic disease. The involved mucosa is white or greyish, thickened, folded and spongy. The genes associated with WSN include mutant cytokeratin keratin 4 (KRT4) and keratin 13 (KRT13). In recent years, new cases of WSN and associated mutations have been reported. Here, we summarise the recent progress in our understanding of WSN, including clinical reports, genetics, animal models, treatment, pathogenic mechanisms and future directions. Gene-based diagnosis and gene therapy for WSN may become available in the near future and could provide a reference and instruction for treating other KRT-associated diseases.

Type
Review Article
Copyright
Copyright © Cambridge University Press 2015 

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