What is a distinguishing feature of Papillon LeFevre syndrome?

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Papillon-LeFevre syndrome is characterized by specific dental and dermatological manifestations, with a noteworthy feature being the premature loss of primary teeth. This condition, resulting from mutations in the CTSC gene, leads to severe periodontal disease and early tooth loss that can occur as early as the primary dentition stage.

The periodontal destruction involves extensive inflammation and attachment loss, ultimately causing teeth to become loose and fall out prematurely. This is a hallmark of the syndrome and is closely associated with the systemic features of the condition.

Other options, while potentially related to dental health, do not represent the core defining characteristics of Papillon-LeFevre syndrome. Frequent orofacial infections can occur but are not exclusive to this condition. The formation of multiple cysts is more pertinent to other dental syndromes, and increased cavity susceptibility, while a concern in general dental health, does not specifically characterize Papillon-LeFevre syndrome. Hence, the premature loss of primary teeth stands out as a distinguishing feature of this genetic disorder.

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