Schnabl Dagmar, Thumm Felix Maximilian, Kapferer-Seebacher Ines, Eickholz Peter
Department of Prosthetic Dentistry, Medical University Innsbruck, 6020 Innsbruck, Austria.
Department of Conservative Dentistry and Periodontology, Medical University Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria.
Healthcare (Basel). 2022 Dec 10;10(12):2505. doi: 10.3390/healthcare10122505.
Papillon-Lefèvre syndrome (PLS) is a rare hereditary disease characterized by palmoplantar hyperkeratosis (PPK) and periodontitis in the primary and permanent dentition, usually resulting in edentulism in youth. Subsiding of PLS-associated periodontitis through specific therapy has occasionally been reported. We aimed to systematically assess periodontal treatment strategies that may decelerate disease progression. A systematic literature search was conducted at PubMed/LIVIVO/Ovid (Prospero registration number CRD42021223253). Clinical studies describing periodontal treatment success-defined as loss of ≤four permanent teeth because of periodontitis and the arrest of periodontitis or probing depths ≤ 5 mm-in individuals with PLS followed up for ≥24 months. Out of the 444 primarily identified studies, 12 studies reporting nine individuals were included. The timely extraction of affected or, alternatively, all primary teeth, compliance with oral hygiene instructions, supra- and subgingival debridement within frequent supportive periodontal care intervals, and-in eight patients-adjunctive systemic antibiotic therapy (mostly amoxicillin/metronidazole) effected a halt in disease progression. The suppression of below the detection limit was correlated with the subsiding of periodontitis. Successful controlling of PLS-associated periodontitis may be achieved if high effort and patient compliance are provided.
掌跖角化-牙周破坏综合征(PLS)是一种罕见的遗传性疾病,其特征为掌跖角化过度(PPK)以及乳牙列和恒牙列的牙周炎,通常会导致患者在年轻时牙齿全部脱落。偶尔有报告称通过特定治疗可使PLS相关的牙周炎得到缓解。我们旨在系统评估可能减缓疾病进展的牙周治疗策略。在PubMed/LIVIVO/Ovid数据库中进行了系统的文献检索(国际前瞻性系统评价注册库注册号CRD42021223253)。纳入了描述牙周治疗成功的临床研究,牙周治疗成功定义为因牙周炎导致的恒牙缺失≤4颗、牙周炎得到控制或探诊深度≤5 mm,研究对象为随访时间≥24个月的PLS患者。在初步检索到的444项研究中,有12项研究报告了9例患者并被纳入。及时拔除患牙或所有乳牙、遵守口腔卫生指导、在频繁的牙周支持治疗期间进行龈上和龈下清创,以及8例患者接受辅助性全身抗生素治疗(主要是阿莫西林/甲硝唑),均使疾病进展停止。牙周炎缓解与炎症指标降至检测限以下相关。如果付出巨大努力并确保患者依从性,PLS相关的牙周炎可能得到成功控制。