Three cases with papillon-lefevre syndrome

Abstract

Papillon-Lefevre Syndrome is an extremely rare genetic disorder that typically effects infants of approximately one to 5 years of age. Papillon-Lefevre Syndrome is characterized by the development of palmar-plantar hyperkeratosis and early loss of the primary (deciduous) and permanent teeth due to rapidly progressive periodontopathy. The primary (deciduous) teeth frequently become loose and fall out by about five years of age. In the general population, the disorder occurs in approximately one to 4 individuals per 1.000.000. Here we present a Papillon- Lefevre Syndrome case, which is rarely seen, with a review of the literature.

Keywords

• Periodontitis Juvenile, Papillon-Lefevre Disease, Keratoderma Palmoplantar

Papillon-Lefevre sendromlu üç olgu

Abstract

Papillon-Lefevre Sendromu, tipik olarak 1-5 yaş civarında ortaya çıkan son derece nadir genetik bir bozukluktur. Papillon-Lefevre Sendromu, palmoplantar hiperkeratoz ile başlayan ve hızla gelişen periodontopatiye bağlı olarak süt dişleri ve kalıcı dişlerin erken kaybıyla karakterizedir. Sütdişleri serbest hale gelir ve bunlar 5 yaş civarında kaybedilir. Genel populasyonda bozukluğun görülme sıklığı, yaklaşık milyonda 1-4 civarındadır. Nadir görülmesi nedeniyle Papillon-Lefevre Sendromlu üç olguyu ilgili literatür bilgileri ile birlikte sunuyoruz.

Keywords

• Juvenile periodontitis, Papillon-Lefevre Sendromu, Palmoplantar hiperkeratoz

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