Long-term follow-up of periodontitis in a patient with Chediak-Higashi syndrome. A case report

Chediak-Higashi syndrome (CHS) is an extremely rare hereditary disease char acterized by leukocyte dysfunction. We report on a 21-year-old woman who pr esented at the age 9 years with CHS and serious periodontal tissue destruct ion around erupted teeth. The patient had received systemic, radiographic, immunological, microbial, and clinical periodontal examinations since child hood. The chemotactic activity of neutrophils in the Boyden chamber assay w as 22% of the control, and leukocyte bactericidal activity was one-third of the control. Actinobacillus actinomycetemcomitans, Porphyromonas gingivali s, and Prevotella intermedia were isolated from periodontal pockets. Period ontal treatment including oral hygiene was provided, followed by profession al tooth cleaning from the age of 12 to 21 years. However the mobility of t eeth and the inflammation of periodontal tissue progressed. This CHS patien t presented with periodontal disease of extremely early onset, which was re sistant to periodontal treatment.