Papillon-lefevere syndrome-Consanguity as a risk factor in siblings (Case series)

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Case Report

Author Details : Shilpa J Parikh, Jaya Dubey*, Jigna S Shah

Volume : 7, Issue : 3, Year : 2021

Article Page : 210-215

https://doi.org/10.18231/j.ijohd.2021.042

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Abstract

Papillon-Lefevere syndrome is a very rare autosomal recessive trait characterized by palmoplantar hyperkeratosis and severe generalized early-onset periodontitis leading to premature loss of primary and permanent dentitions. Various etiopathogenesis factors are associated with syndrome. The palmoplantar keratoderma typically has its onset between the ages of 1 and 4 years and severe periodontitis starts at age of 3-4 years. A dentist plays an important role in early diagnosing and preserving remaining teeth in oral cavity. This case series describes three cases of PLS in siblings with consanguineously married parents. All siblings in the family were affected which makes this a rare case.

Keywords: Consanguineous, Palmoplantar keratosis, Periodontitis, Cathepsin­ C gene

How to cite : Parikh S J, Dubey J, Shah J S, Papillon-lefevere syndrome-Consanguity as a risk factor in siblings (Case series). Int J Oral Health Dent 2021;7(3):210-215

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