Behavior of neutrophilic granulocytes in a case of Papillon-Lefèvre syndrome

Recently we had the opportunity to examine and follow up over a period of 2 years an unusual case of Papillon-Lefèvre syndrome (PLS). A 10 year old boy exhibited all symptoms typical of PLS except periodontitis and premature loss of deciduous teeth. The present report aimed at studying the functional capacity of his neutrophilic granulocytes. It integrates clinical observations, histopathological findings and results of in vitro tests. The bioptic material examined included one gingival biopsy, extracted teeth, suppurative material discharged from periodontal pockets, pus emanating from a mucosal abscess, and peripheral blood leucocytes. The neutrophils were sampled on two separate occasions in two independent laboratories and tested for a variety of functions, i.e. motility, random and directional locomotion (chemotaxis), phagocytosis, membrane potential depolarization, oxygen consumption, NBT reduction, and intracellular killing of bacteria and fungi. Findings and test data indicated that in this case of PLS, neutrophilic granulocytes behaved normally with respect to all these functions including margination in blood vessels, emigration, phagocytosis of a broad range of bacteria, degranulation of lysosomes, and intracellular destruction. The data imply that factors other than neutrophil defects may be responsible for rapidly destructive periodontitis in cases where PLS is not associated with an increased susceptibility to infection.