E. Firatli et al., CLINICAL AND IMMUNOLOGICAL FINDINGS IN 2 SIBLINGS WITH PAPILLON-LEFEVRE SYNDROME, Journal of periodontology, 67(11), 1996, pp. 1210-1215
RAPID AND SEVERE DESTRUCTION Of periodontal tissues in early childhood
has been reported both in systemically healthy children and in childr
en with systemic disorders. In this study, the clinical and immunologi
cal findings of two siblings in a family with Papillon-Lefevre syndrom
e are presented. The peripheral blood lymphocytes were analyzed using
a double colored flow cytometry and adequate monoclonal antibodies to
CD2, CD3, CD4, CD5, CD8, CD11b, CD16, CD19, and HLA-DR receptors. CD11
b expression was found to be higher in both siblings (35% and 37%). Th
e elevated CD11b expression may be related to a defect in neutrophils.
The expression of natural killer cells was found to be higher in one
patient but the results were in normal range. The CD2+, CD3+, CD4+, CD
5+, CD8+, and CD19+ lymphocytes were in normal range in both patients.
We think that the depressed chemotaxis of peripheral neutrophils, and
higher expression of HLA-DR and CD11b molecules in peripheral leukocy
tes were useful in explaining the pathogenesis of the Papillon-Lefevre
syndrome.