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Case Reports
. 2019 Apr 21:2019:4210347.
doi: 10.1155/2019/4210347. eCollection 2019.

Papillon-Lefèvre Syndrome: Diagnosis, Dental Management, and a Case Report

Jean-Claude Abou Chedid  1 , Michel Salameh  1 , Abbass El-Outa  2 , Ziad E F Noujeim  3
Affiliations
Case Reports

Papillon-Lefèvre Syndrome: Diagnosis, Dental Management, and a Case Report

Jean-Claude Abou Chedid et al. Case Rep Dent. .

Abstract

Aim: This paper revisits Papillon-Lefèvre syndrome (PLS), addresses its diagnostic update and dental management, and reports a case of a 5-year-old Lebanese patient with consanguineously married parents.

Background: PLS, also known as "keratoris palmoplantaris with periodontopathia" and "hyperkeratosis palmoplantaris with periodontosis," is an extremely rare autosomal-recessive trait that combines a diffuse palmoplantar hyperkeratosis and a severe generalized, progressive prepubertal form of a precocious form of juvenile, aggressive periodontitis.

Case description: We are reporting a 5-year-old boy that sustained a spontaneous loss of all his primary teeth. At consultation, he was under treatment for hyperkeratosis of his palms and soles. Detailed family history of the child revealed that the patient's parents, grandparents, and relatives were consanguineously married and two of his cousins displayed similar clinical signs (palmoplantar hyperkeratosis and premature loss of deciduous and most of the permanent teeth).

Conclusion: PLS is an extremely rare disorder that usually becomes apparent from approximately 1-5 years of age. Genetic counseling should always be suggested to parents of affected children, informing them of chances of their offspring having the inherited disease.

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Figures

Figure 1
Figure 1
(a and b) Intraoral maxillary and mandibular aspects before multiple dental extractions. (c) Panoramic radiograph before extractions.
Figure 2
Figure 2
Hyperkeratosis on hand palms (a and b) and dorsal surfaces (c).
Figure 3
Figure 3
Hyperkeratosis of soles of feet (scaly and rough soles).
Figure 4
Figure 4
(a) Maxillary and mandibular removable complete dentures. (b) Insertion of maxillary denture in the mouth.
Figure 5
Figure 5
The erupting mandibular central incisors.
See this image and copyright information in PMC

References

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