Research Progress of Papillon-Lefèvre Syndrome.

doi:10.13701/j.cnki.kqyxyj.2018.09.002

Abstract

Abstract: The Papillon-Lefèvre syndrome (PLS, OMIM 245000) is a rare autosomal recessive disorder characterized by a diffuse palmoplantar hyperkeratosis and rapid progressive periodontal devastation. Most researchers consider that PLS was caused by the mutations on the cathepsin C (CTSC) gene. Some studies indicate that immune factors, microbial factor, and other genetic factor participate in the process as well. However, the exact cause is still unknown. Most of the articles are case reports, and there are not enough researches on etiology and pathogenesis. This paper reviews the etiology, pathogenesis, clinical presentation, and treatment modalities of the disease.

Key words: Papillon-Lefèvre syndrome, Mutation , Etiology, Pathogenesis, Periodontal treatment

WU Yue-lin, ZHAO Lei, WU Ya-fei.. Research Progress of Papillon-Lefèvre Syndrome.[J]. Journal of Oral Science Research, 2018, 34(9): 928-931.

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