Idon P I, Olasoji H O, Fusami M A
Department of Dental Surgery, University of Maiduguri Teaching Hospital, Maiduguri, Borno State.
Niger Postgrad Med J. 2015 Mar;22(1):75-82.
The objective of this paper is to give a review of the literature on the aetiopathogenesis, clinical presentation, differential diagnosis and treatment of Papillon-Lefevre Syndrome, and present three cases of PLS in siblings of the same parents demonstrating typical clinical and radiological features of the condition.
Three cases of Papillon-Lefevre syndrome affecting three girls among six siblings of normal parents who are first-degree cousins. The three siblings aged 14, 6 and 4 ⅇ years all had severe gingivitis, periodontitis with grossly mobile teeth and tooth migration. Other findings included premature exfoliation of deciduous teeth (2/3), premature loss of permanent teeth in one sibling. Plantar hyperkeratosis was detected in all of the cases, leading to a difficult and painful walking in two cases due to lesions on the soles. Palmar hyperkeratosis was present in the oldest sibling as well. Radiologic study confirmed alveolar bone destruction in two cases.
Early recognition of this entity as well as multidisciplinary management may help in the prognosis of these cases.
本文旨在综述关于帕皮永 - 勒费弗尔综合征（Papillon-Lefevre Syndrome）的病因发病机制、临床表现、鉴别诊断及治疗的文献，并呈现三例来自同一对父母的患有帕皮永 - 勒费弗尔综合征（PLS）的兄弟姐妹病例，展示该病典型的临床和放射学特征。
三例帕皮永 - 勒费弗尔综合征病例，累及一对表亲父母的六个孩子中的三个女孩。这三个兄弟姐妹年龄分别为14岁、6岁和4岁，均患有严重的牙龈炎、牙周炎，牙齿严重松动且移位。其他发现包括乳牙过早脱落（2/3），其中一个兄弟姐妹恒牙过早缺失。所有病例均检测到足底角化过度，两例因足底病变导致行走困难且疼痛。年龄最大的兄弟姐妹也存在手掌角化过度。放射学研究证实两例存在牙槽骨破坏。
早期识别该疾病以及多学科管理可能有助于改善这些病例的预后。
