A Possible Late Onset Variation of Papillon‐Lefèvre Syndrome: Report of 3 Cases

Abstract

Three unusual cases of Papillon-Lefèvre syndrome with late onset of features and relatively mild periodontal disease are presented. These examples confirm some of the late onset of features and mild periodontal presentation of a previous case report. Bacteriologic associations, polymorphonuclear leukocyte chemotactic, phagocytic, and bactericidal activity, and therapeutics with regard to this syndrome are discussed.

Papillon Lefevre Syndrome: a case report with review

“…Bullon et al in 1993 documented a case report of 2 sisters exhibiting early onset palmoplantar hyperkeratosis and late onset periodontitis [32]. Brown et al in 1993 described 3 cases having late onset periodontitis as well as skin manifestations [33].…”

Neutrophil Elastase, Proteinase 3, and Cathepsin G as Therapeutic Targets in Human Diseases

“…The exact cause of periodontal disease in PLS has not been elucidated, but it has been attributed to a neutrophil defect resulting in decreased phagocytosis and chemotaxis (Brown et al, 1993;Bullon et al, 1993;Firatli et al, 1996). Association of neutrophil or myeloid abnormalities with severe periodontal destruction and the development of rapid periodontal infections in experimentally induced neutropenia in animals suggest that neutrophils are protective against periodontal destruction.…”

Cytokine production by leukocytes of Papillon–Lefèvre syndrome patients in whole blood cultures

“…Some case reports described favorable results after early extraction of all erupted teeth, followed by an edentulous period, to prevent subsequent infection of the non-erupted teeth [10,11,[35][36][37]. However, successful cases in which A. actinomycetemcomitans had either not been detected before treatment or had been eliminated by treatment have also been described that did not include large-scale extractions [7,9,32,[38][39][40]. Success of periodontal therapy in PLS may be related to infection with A. actinomycetemcomitans and its elimination, respectively.…”