Neutrophil dysfunction in prepubertal periodontitis associated with Papillon-Lefèvre syndrome

Although prepubertal periodontitis (PP) is often associated with the Papillon-Lefèvre (PLS), the etiopathogenetic mechanism in this disease has not been clarified as yet. A three and half year old female with leukocyte chemotaxis deficiency and decrease of T-lymphocytes is presented. The immunohistochemical evaluation of neutrophils showed a reduction of myeloperoxidase content, whereas ultrastructural studies revealed no particular change. The possible role of such a deficiency in the pathogenesis of the periodontal lesion is discussed.