Neutrophil Function in 8 Cases of Papillon-Lefevre Syndrome

Statement of Problem: Papillon Lefevre syndrome (PLS) is a rate autosomal recessive disorder, which is characterized by palmar- plantar hyperkeratosis and rapid periodontal destruction of primary and permanent dentitions. Purpose: The aim of the present study was to evaluate the peripheral blood neutrophil function including random locomotion, chemotaxis and oxidative mechanism of killing in a group of patients with PLS. Materials and Methods: Peripheral blood was obtained from 8 PLS patients and 92 healthy control subjects. PMN mobility was measured by a modification of the micromethod of Addison and Babbage. Latex-Stimulated NBT reduction test described by Park et al was followed. Data were analyzed by Mann Whitney U test. Results: The chemotactic activity in the PLS group was significantly lower than control group (89.5±21.6 vs 113±16 mm, P<0.002). The rate of NBT reduction by PLS patients leukocytes was 50.6±14.9% in comparison with the control group (52.2±16.1%). The patients group showed a random locomotion rate of 46.5±10.4 mm. This value for the control group was 43.9±13.6 mm. Both oxidative mechanism of killing and random location were not significantly different from those of the healthy control subjects (P>0.05). Conclusion: The present study indicated an impaired neutrophil chemotaxis in PLS patients.