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Case Reports
. 2011 Sep;15(3):352-7.
doi: 10.4103/0973-029X.86724.

Papillon-Lefévre syndrome

Bagavad Gita Veerabahu  1 , Sc Chandrasekaran, Mohammed Nazish Alam, Mahalakshmi Krishnan
Affiliations
Case Reports

Papillon-Lefévre syndrome

Bagavad Gita Veerabahu et al. J Oral Maxillofac Pathol. 2011 Sep.

Abstract

The goal of periodontal therapy has always been regeneration of the lost tissues. However, conventional periodontal therapy has not always been successful in achieving regeneration, especially when it is part of a syndrome. This case report involves a 13-year old male patient with the chief complaint of mobile teeth for over 3 months. His dental history revealed early loss of primary dentition, around 3-4 years of age and that he noticed mobility of permanent incisors and molars at 9-10 years. Keratotic skin lesions on the palms and soles were present since the age of 3 years. Full mouth intra-oral periapical radiographs showed extensive bone loss upto apical thirds of the teeth and an orthopantamograph showed "floating in air" appearance. Further, a lateral cephalogram was taken to rule out any calcifications of the duramater. The case was provisionally diagnosed to be Papillon Lefévre syndrome. A conventional polymerase chain reaction assay was also done to assess the virulence genes in aggressive periodontitis. Though the management of PLS involves the regular phases of periodontal therapy, namely, etiotropic, surgical, restorative and maintenance phases, the complete esthetic and functional rehabilitation also involves other specialities especially prosthodontic and dermatologic and later an implantologist. After appropriate periodontal and prosthodontic management, the patient has been followed up for over a year and is maintaining in a stable condition.

Keywords: Generalized aggressive periodontitis; Palmoplantar hyperkeratosis; Papillon-Lefévre syndrome; neutrophil function tests; polymerase chain reaction.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Facial view of patient
Figure 2
Figure 2
Hyperkeratotic patch on fingers
Figure 3
Figure 3
Hyperkeratotic patches on kness
Figure 4
Figure 4
Lesions on feet
Figure 5
Figure 5
Facial view of lower anterior teeth showing gingival recession and inflammation
Figure 6
Figure 6
Palatal view of upper anterior teeth
Figure 7
Figure 7
Lingual aspect of lower anteriors
Figure 8
Figure 8
Full mouth intraoral periapical radiographs showing bone loss up to apical one-thirds of teeth
Figure 9
Figure 9
Orthopantamograph of patient showing “floating in air” appearance
Figure 10
Figure 10
Lateral cephalogram showing no evidence of intracranial calcifications
Figure 11
Figure 11
Facial view after restoration with a flexible partial denture, replacing the missing teeth
See this image and copyright information in PMC

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