Periodontal involvement in leukocyte adhesion deficiency: review of the literature and a case report

  • Nasim Seyfi Department of Pedodontics, Dental School, Golestan University of Medical Sciences, Gorgan, Iran.
  • Mehrnoosh Sabzeghabaie Periodontist, Tehran, Iran.
  • Sara Dehghankhalili Department of Pediatric Dentistry, AJA University of Medical Sciences, Tehran, Iran.
Keywords: diagnosis, leukocyte adhesion deficiency syndrome, periodontitis

Abstract

Objective Leukocyte adhesion deficiency (LAD) is a rare, autosomal recessive inherited disorder; LAD-I which is the most common type, occurs due
to mutations in the CD18 gene. This mutation down-regulates the expression of ß2 integrin leukocyte cell surface molecules, which are necessary
for the adhesion of leukocytes to endothelial cells, transendothelial migration, and chemotaxis. The major symptoms are recurrent severe bacterial
infections without pus formation, recurrent or progressive necrotizing soft tissue infections, marked leukocytosis and severe progressive
periodontitis accompanied by alveolar bone loss, periodontal pockets, and partial or total early loss of primary and permanent teeth.
Cases Herein, we report a case of moderate LAD-I in a nine year-old boy with severe alveolar bone loss and aggressive periodontitis. For
several years, approximately every month, the patient was followed up to evaluate the status of primary and permanent dentition. During
these visits, scaling and root planing were performed and severely mobile teeth were extracted. The patient is now under regular follow up.
Conclusion This case confirms the need for interactions between the medical groups to identify and manage medically compromised
children with rare diseases. It is important to include LAD in the differential diagnoses of children presenting with periodontal disease. Early
correct diagnosis of LAD has various benefits for patients.
 

Published
2016-06-06
Section
Case Report