Sreeramulu Basapogu, Shyam Naragani Dvn, Ajay Pilla, Suman Pathipaka
Department of Prosthodontics, Government Dental College and Hospital, Hyderabad, Telangana State, India.
Department of Oral Pathology, Government Dental College and Hospital, Hyderabad, Telangana State, India.
Clin Cosmet Investig Dent. 2015 Jul 15;7:75-81. doi: 10.2147/CCIDE.S76080. eCollection 2015.
Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age. Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria. Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth. Here, we are presenting an elaborate review of PLS, its etiopathogenesis, clinical presentation, and management options.
帕皮永-勒费夫尔综合征(PLS)是一种罕见的常染色体隐性疾病,其特征为弥漫性掌跖角化病和早发性侵袭性牙周炎,导致在非常年轻的时候乳牙和恒牙过早缺失。多种致病因素与该综合征相关,如免疫改变、基因突变和细菌的作用。由于存在诸如早期牙周破坏和恒牙早萌等特征性表现,牙医在PLS的诊断和管理中发挥着重要作用。在此,我们对PLS及其发病机制、临床表现和管理方案进行详尽综述。