Papillon-Lefèvre syndrome: from then until now

Faiez N Hattab

doi:10.20517/2573-0002.2018.22

Abstract

Papillon-Lefèvre syndrome: from then until now

Highlights

In 1924, the French physicians Papillon and Lefévre described a brother and sister; the products of first cousin matching, with condition characterized by palmoplantar hyperkeratosis (PPK) and severe early-onset periodontitis[1]
Papillon-Lefévre syndrome (PLS) is very rare disorder inherited in autosomal recessive pattern; that is, both parents are phenotypically healthy and there is no family history of the disease, other than the affected person
The two cardinal features of PLS are PPK and severe early onset of destructive periodontitis leading to premature loss of both primary and permanent dentitions

Summary

Introduction

In 1924, the French physicians Papillon and Lefévre described a brother and sister; the products of first cousin matching, with condition characterized by palmoplantar hyperkeratosis (PPK) and severe early-onset periodontitis[1]. Gorlin et al.[2] reviewed 46 Papillon-Lefévre syndrome (PLS) cases described in the literature. They added calcification of the falx cerberi to the syndrome, converting it into a triad. In review of 124 PLS cases, Haneke[3] concluded that: (1) males and females were affected; (2) there was no racial predominance of the condition; (3) consanguinity is a feature in one-third of the cases; and (4) an increased susceptibility to infection, beside periodontitis, in 25% of PLS patients

Objectives

Methods

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Discussion

Conclusion