Neutrophil Function in 8 Cases of Papillon-Lefevre Syndrome

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.

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.

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.

The chemotactic activity in the PLS group was significantly lower than control group (89.5±21.6 vs 113±16 m, 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 m. This value for the control group was 43.913.6 m. Both oxidative mechanism of killing and random location were not significantly different from those of the healthy control subjects (P>0.05).

The present study indicated an impaired neutrophil chemotaxis in PLS patients.