Fageeh Hytham N
Diplomate and Vice Dean, Division of Periodontics, Department of Preventive Dental Sciences, College of Dentistry, Jazan University, Jazan, Kingdom of Saudi Arabia.
Int J Clin Pediatr Dent. 2018 Jul-Aug;11(4):352-355. doi: 10.5005/jp-journals-10005-1538. Epub 2018 Aug 1.
Papillon-Lefèvre is an autosomal recessive syndrome that starts in early periods of childhood. Characteristic features include palmar plantar hyperkeratosis, aggressive periodontal disease, and a tendency for dry and chopped skin, thin and sparse hair. Patients show signs of premature tooth loss at the age of 2 to 4 years, which is then followed by the loss of permanent dentition during adolescence. The presence of both skin and oral lesions in this syndrome differentiates this unusual genodermatosis from other pathology of palmoplantar keratoderma (PPK). The etiopathogenesis of this syndrome is somewhat obscure; however, immunologic, genetic, and possible bacterial etiologies have been proposed. The dental practitioner is often the first to diagnose the disease, as there is a significant degree of periodontal breakdown that is involved at an early age. This report presents a clinical presentation of two brothers detected with Papillon-Lefèvre syndrome (PLS). Fageeh HN. Papillon-Lefèvre Syndrome: A Rare Case Report of Two Brothers and Review of the Literature. Int J Clin Pediatr Dent 2018;11(4):352-355.
帕皮永 - 勒费夫尔综合征是一种常染色体隐性综合征，始于儿童早期。其特征包括掌跖角化过度、侵袭性牙周病，以及皮肤干燥皲裂、头发稀疏的倾向。患者在2至4岁时出现乳牙过早脱落的迹象，随后在青春期恒牙也会脱落。该综合征中皮肤和口腔病变的同时存在，使其与其他掌跖角化病（PPK）的病理情况有所不同。此综合征的病因发病机制尚不清楚；然而，已经提出了免疫、遗传以及可能的细菌病因。牙科医生常常是最早诊断出该疾病的，因为在早期就存在严重的牙周破坏。本报告展示了两名被检测出患有帕皮永 - 勒费夫尔综合征（PLS）的兄弟的临床表现。法吉 HN。帕皮永 - 勒费夫尔综合征：两兄弟的罕见病例报告及文献综述。《国际临床儿科牙科学杂志》2018年；11（4）：352 - 355。
