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[Papillon-Lefèvre syndrome: A new case].

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie - 1 Apr 2017

Martinho S, Levade T, Fergelot P, Stephan J-L

Abstract excerpt

Papillon-Lefèvre syndrome (PLS) is a rare primary immunodeficiency, which combines severe periodontal disease with edentulism and palmoplantar keratosis (PPK). PLS is inherited as an autosomal recessive trait and is due to mutations in the cathepsin C gene. The biological properties of the neutrophils (PN) are altered, leading to a gingival dysbiosis and bacterial overgrowth, with intense inflammation of the...

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PMID: 28242153
DOI: 10.1016/j.arcped.2017.01.013

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

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