Shah Jigna, Goel Shweta
Department of Oral Medicine and Radiology, G.D.C.H, Ahmedabad, Gujarat - 380 016, India.
Indian J Dent Res. 2007 Oct-Dec;18(4):210-3. doi: 10.4103/0970-9290.35834.
Papillon-Lefevre syndrome is a rare autosomal recessive disorder in which there is palmoplantar keratinization and premature loss of both deciduous and permanent teeth. The palmoplantar keratoderma typically has its onset between the ages of 1 and 4 years and severe periodontitis starts at the age of 3 or 4 years. An early diagnosis of the syndrome can help preserve the teeth by early institution of treatment, using a multidisciplinary approach. We present two cases of the syndrome having all of the characteristic features.
掌跖角化-牙周破坏综合征是一种罕见的常染色体隐性疾病,其特征为掌跖角化以及乳牙和恒牙过早缺失。掌跖角化病通常在1至4岁之间发病,严重的牙周炎在3或4岁时开始。通过多学科方法早期诊断该综合征并尽早开始治疗,有助于保留牙齿。我们报告两例具有所有特征性表现的该综合征病例。