Papillon-Lefevre Syndrome. Analysis of neutrophil chemotaxis
The neutrophil is the key protective cell in the defense of periodontal tissues. Neutrophil functions in Papillon-Lefevre Syndrome (PLS) have been evaluated previously from case reports. The data from the previous studies varied from normal neutrophil chemotaxis to decreased chemotaxis. This study was designed to assess the peripheral blood neutrophil chemotaxis in a group of patients with PLS.
Seven patients with a mean age of 10.56 years were followed in the study. All of the patients revealed extensive loss of soft tissue attachment and alveolar bone, and mobility and migration of teeth. Radiographs showed extensive loss of alveolar bone around affected teeth. Samples of heparinized blood were taken from each patient and the healthy controls for neutrophil extraction. The neutrophil chemotaxis was analyzed using the zymosan activity serum assay (ZAS). Chemotaxis and spontaneous migration measurements were compared with those of the healthy control subjects.
The results showed that the chemotaxis and spontaneous migration values of the PLS subjects were significantly decreased when compared with the controls.
Several case reports discussing neutrophil functional defects have been reported. This study is the first attempt to test the hypothesis in a statistical manner and generalize the data. The decreased chemotaxis of peripheral blood neutrophils strongly suggests that the neutrophils may act as one of the important determinants of the pathogenesis of severe periodontal destruction in patients with PLS. [D.S.S.]
Firatli, E., B. Tuzun, and A. Efeoglu, J Periodont, 67:617,1996