Clinical and immunological findings in 2 siblings with Papillon-Lefèvre syndrome

Journal of Periodontology
E FiratliS Badur

Abstract

Rapid and severe destruction of periodontal tissues in early childhood has been reported both in systemically healthy children and in children with systemic disorders. In this study, the clinical and immunological findings of two siblings in a family with Papillon-Lefèvre syndrome 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. CD11b expression was found to be higher in both siblings (35% and 37%). The 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+, CD5+, 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 leukocytes were useful in explaining the pathogenesis of the Papillon-Lefèvre syndrome.

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Citations

Feb 24, 2001·Pediatric Dermatology·S Khandpur, B S Reddy
Oct 10, 2009·Dermatology : International Journal for Clinical and Investigative Dermatology·Mazen KurbanAngela M Christiano
Oct 12, 2012·Journal of Indian Society of Periodontology·Fayiza Yaqoob KhanMubashir Mushtaq
Nov 23, 2005·Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics·Faiez N Hattab, Wala M Amin
Jul 10, 2004·International Journal of Paediatric Dentistry·S Patel, L E Davidson
Oct 20, 2004·Journal of Clinical Periodontology·T Lundgren, S Renvert
Sep 15, 2006·Journal of Clinical Periodontology·B SchacherP Eickholz
Apr 21, 2005·Journal of Dental Research·T LundgrenD N Tatakis
Jun 10, 2006·Journal of Pediatric Ophthalmology and Strabismus·Pinar SaatciSalih Kavakçu

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