Papillon Lefevre Syndrome: a case report with review

Bhawandeep Kaur

doi:10.4172/2161-1122.1000156

Abstract

Papillon-Lefevre syndrome is a rare autosomal recessive genetic disorder. The clinical manifestations include palmer planter hyperkeratosis with precocious progressive periodontal disease that results in premature exfoliation of primary and permanent dentitions. Patients are often edentulous at an early age.

Highlights

Papillon lefevre is a rare genodermal condition characterized firstly by two French Physicians Papillon and Lefevre in 1924, in a brother and sister suffering from palmoplantar hyperkeratosis associated with early onset periodontitis and pre mature loss of deciduous as well as permanent dentition [1]
It may be attributed to high rate of consanguineous marriages in Arab communities [8,9]
The purpose of this paper is to demonstrate clinical as well as radiological features of Papillion Lefevre Syndrome

Summary

Introduction

Papillon lefevre is a rare genodermal condition characterized firstly by two French Physicians Papillon and Lefevre in 1924, in a brother and sister suffering from palmoplantar hyperkeratosis associated with early onset periodontitis and pre mature loss of deciduous as well as permanent dentition [1]. Gorlin et al in 1964 [2] have added the third component of dural calcification for making diagnosis of this syndrome. It has a prevalence of 1-4 cases per million in general population and the carrier frequency appears to be 2-4 per thousand population with no sexual predominance [3,4,5,6]. The skin lesions are thought to be because of disturbances in ectodermal and mesodermal components but there is no reason to explain the rapid loss of all the deciduous as well as permanent teeth in the order of their eruption [10]

Objectives

Discussion

Conclusion