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This paper aims to highlight the detrimental periodontal condition in patients with Papillon-Lefèvre syndrome
(PLS) and the pivotal role of a Periodontist in the diagnosis of the condition. PLS is also known as
palmo-plantar keratosis (PPK) with an unusual periodontal status. Uncontrolled early-onset periodontitis,
which affects both primary and permanent dentitions alike, is a hallmark of the syndrome. PLS patients
exhibit defected neutrophil chemotactic function due to substandard activity of Cathepsin C (CTSC) gene.
The result is the failure of elimination of periodontal pathogens that leads to severe periodontal destruction.
We report a case of an 11 years old Pakistani girl affected with PLS whose parents are consanguineously
married. Since PLS is a rare autosomal recessive disorder and multiple consanguinity in a family, increases the
risk of the occurrence of the syndrome in the off springs, the case report also highlights the importance of
pre-marital genetic mapping and conception counseling for the families.
Ayesha Hanif, Zaheer Hussain Chachar, Haroon Rashid Baloch. (2020) Papillon-Lefèvre Syndrome: Detrimental Periodontal Condition in the Affected Individuals, The Pakistan Journal of Medicine and Dentistry, Volume 9, Issue-2.
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