Papillon‐Lefèvre Syndrome. Analysis of Neutrophil Chemotaxis

Abstract

Papillon‐Lefèvre syndrome (PLS) is described as the association of palmar‐plantar hyperkeratosis with precocious periodontal disease which results in exfoliation of primary and permanent dentitions. This study was planned to assess the Chemotaxis of peripheral blood neutrophils in 7 patients (3 females and 4 males) with Papillon‐Lefèvre syndrome. The neutrophil Chemotaxis was analyzed using the zymosan activated serum (ZAS) assay. Chemotaxis and spontaneous migration measurements were compared to those of the healthy control subjects. The peripheral blood neutrophil Chemotaxis and spontaneous migration were depressed in all patients with Papillon‐Lefèvre syndrome. The decreased Chemotaxis of peripheral blood neutrophils strongly suggests that the neutrophils may act as one of the important key determinants in the pathogenesis of severe periodontal destruction in patients with PLS. J Periodontol 1996;67:617–620.

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.…”

Role of Polymorphonuclear Leukocyte-Derived Serine Proteinases in Defense againstActinobacillus actinomycetemcomitans

“…Case studies on PLS patients in the last 25 years report contradictory findings. Several studies reported a depressed in vitro PMN chemotaxis (9,10,23,24,45,68,70,74), whereas others reported normal values (10,47,68,73). Similar conflicting results were found for phagocytosis (6,7,9,10,26,27,62,73), the capacity to kill S. aureus (6,10,54,62,70), and the production of hydrogen peroxide (2,5,6,10,42,55,62,68,71).…”

Congenital Defects in Neutrophil Dynamics

“…Among others, CTSC targets are the neutrophil effectors NE, cathepsin G, and proteinase-3 [108, 109]. Increased susceptibility to infections in some cases [110] and neutrophil chemotaxis deficiency was reported in PLS patients [111]. It is controversial whether neutrophil chemotaxis is intrinsically defective in CTSC-deficient neutrophils.…”