Papillon-Lefèvre syndrome: A case report of two siblings and review of the literature
DOI:
https://doi.org/10.66344/jpad.24.1.2014.182Keywords:
Papillon-Lefèvre syndrome, periodontitis, palmoplantar keratodermaAbstract
Papillon-Lefèvre syndrome is a rare autosomal recessive genodermatosis which is characterised by periodontitis, palmoplantar keratoderma and predisposition to pyogenic infections and occurs due to cathepsin C gene mutation (located on chromosome11).The loss of primary teeth usually occurs by the age of 4 years and secondary teeth by second decade. The disorder is associated with significant cosmetic and functional disability.References
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Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth. J Pediatr. 1964;65:895-908.
Bach JN, Levan NE. Papillon-Lefèvre syndrome. Arch Dermatol. 1968;97:154-8.
Ullbro C, Crossner CG, Nederfors T et al. Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome. J Am Acad Dermatol. 2003;48:345-51.
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Siragusa M, Romano C, Batticane N et al. A new family with Papillon-Lefèvre syndrome: effectiveness of etretinate treatment. Cutis. 2000;65:151-5.
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