Generalized periodontitis associated with Chédiak-Higashi syndrome.

BACKGROUND

Chédiak-Higashi syndrome (CHS) is a rare immunodeficient disorder. Patients with CHS are prone to severe periodontitis. To date, limited improvement following periodontal therapy has been reported. Thus, successful clinical outcomes in patients with CHS are of interest.

METHODS

A 12-year-old girl was referred to the Department of Pediatric Dentistry, Hôtel-Dieu/Garancière Hospital, for acute gingival inflammation and periodontal destruction. After a periodontal examination, the patient was sent to the Department of Medicine, Robert Debré Hospital, for a hematologic examination and was diagnosed with CHS. She has been receiving medical and dental treatments since that time. The medical treatment consisted of continuous, long-term antibiotherapy. Supportive periodontal therapy was initiated with 4-month recall periods. We report the diagnosis process and the 9-year follow-up.

RESULTS

Radiographs and a periodontal examination showed deep probing pockets and extensive alveolar bone resorption. Hematologic and immunologic investigations showed normal values. Peripheral blood smears showed giant granules in neutrophils and leukocytes characteristic of CHS. Clinical improvement was observed after the initial periodontal therapy. No periodontitis recurrence was noted over a period of 9 years.

CONCLUSIONS

This case report shows that it is possible to treat periodontitis and maintain the periodontal health of a patient with a mild CHS phenotype over a long period. Patient compliance, regular dental follow-ups, and long-term systemic antibiotic treatments may be useful in stabilizing the periodontal condition of patients with CHS. Dentists must be aware that aggressive periodontitis, combined with general clinical signs, in young patients may reflect rare systemic disorders requiring biologic investigation.