Periodontal Involvement in Leukocyte Adhesion Deficiency: Review of the Literature and a Case Report

Objective: Leukocyte adhesion deficiency(LAD) is a scarce, autosomal recessive inherited disorder . LAD-I which is the most common type occurs due to mutations on the CD18 gene. This mutation leads to absence or severe reduction of leukocyte cell surface expression of ß2 integrin molecules which are necessary for the adhesion of the leukocytes to the endothelial cells, transendothelial migration, and chemotaxis. The major symptoms are recurrent severe bacterial infection without pus formation, recurrent or progressive necrotic soft tissue infections, marked leukocytosis, severe progressive periodontitis accompanied by the alveolar bone loss, periodontal pockets, and partial or total early loss of the primary and permanent dentitions.  Methods: We report a case of moderate LAD-I in a 9-year-old boy with severe alveolar bone loss and aggressive periodontitis. The case confirms the need for interactions between the medical groups to identify and manage these medically compromised children with rare diseases. Results: For several years, approximately every month, the patient was followed up to evaluate the status of the primary and permanent dentition. During these visits, scaling and root planning was performed and severely mobile teeth were extracted.  The patient is now under regular follow up. As healing has improved in the recent years, bone marrow graft is not yet prescribed and follow up should be continued. Conclusion: It is important to include LAD in the differential diagnosis of children presenting with periodontal disease. Early, appropriate diagnosis of LAD provides various benefits for the patients. Key words: Leukocyte adhesion deficiency syndrome, Periodontitis, Diagnosis.