Papillon-Lefevre syndrome: a case report and review of the literature

RESUMO

Papillon-Lefevre syndrome is a rare autosomal recessive syndrome associated with palmoplantar keratoderma and early onset periodontal disease that results in loss of the teeth. Actinomyces Actinomycetemcomitans causing periodontal damage, and alterations in the polymorphonuclear leukocyte function have been postulated or probable pathogenetic mechanism. Early recognition of this entity as well as a multidisciplinary management may help in the prognosis of these cases