Sachdeva Shabina, Kalra Namita, Kapoor Pranav
MDS. Formerly: Senior Resident, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025.
MDS. Professor & Head, Department of Dentistry, University College of Medical Sciences, New Delhi-110095.
J Clin Exp Dent. 2012 Feb 1;4(1):e77-81. doi: 10.4317/jced.50594. eCollection 2012 Feb.
Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis. The etiopathogenesis of the syndrome is relatively obscure and immunologic, genetic or possible bacterial etiologies have been proposed. Due to the vast degree of periodontal breakdown involved at such an early age, the dental surgeon is often the first to diagnose the syndrome. This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lefèvre Syndrome. Key words:Papillon-Lefèvre Syndrome, palmoplantar keratoderma, rapidly progressing periodontitis.
掌跖角化-牙周破坏综合征（PLS）是一种罕见的常染色体隐性疾病，于1924年由两位法国医生帕皮永（Papillon）和勒费夫尔（Lefèvre）首次描述。该疾病的特征是弥漫性掌跖角化病和早熟性侵袭性牙周炎，导致乳牙和恒牙在非常年轻时过早脱落。皮肤病变通常与口腔内表现同时出现，包括手掌和脚底的角化斑块，从轻度银屑病样鳞屑皮肤到明显的角化过度不等。该综合征的病因发病机制相对模糊，有人提出了免疫、遗传或可能的细菌病因。由于在如此早期就涉及广泛的牙周破坏，牙科医生往往是最早诊断出该综合征的人。本文介绍了一名被诊断为掌跖角化-牙周破坏综合征的15岁男性的临床表现。关键词：掌跖角化-牙周破坏综合征；掌跖角化病；快速进展性牙周炎
