Papillon-Lefèvre syndrome: A case report of two siblings and review of the literature

Peerzada Sajad, Iffat Hassan, Syed Imtiyaz

Abstract


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.


Keywords


Papillon-Lefèvre syndrome, periodontitis, palmoplantar keratoderma

Full Text:

PDF

References


Haneke E. The Papillon-Lefèvre syndrome: Keratosis palmoplantaris with periodontopathy. Hum Genet. 1979;51:1-35.

Hart TC, Shapira L. Papillon-Lefèvre syndrome. Periodontol. 2000. 1994;6:88-100.

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.

Levoy, Wollnier S, Hachem-Zadeh S. Immunological study of patients with Papillon-Lefèvre syndrome. Clin Exp Immunol. 1980;40:407-10.

Djawezi D. Deficient phagocytic function in Papillon-Lefèvre syndrome. Clin Exp Immunol. 1980;40:407-10.

Siragusa M, Romano C, Batticane N et al. A new family with Papillon-Lefèvre syndrome: effectiveness of etretinate treatment. Cutis. 2000;65:151-5.


Refbacks

  • There are currently no refbacks.


ISSN: 1560-9014