Periodontitis Associated with Chediak-Higashi Syndrome in a Young African American Male

Authors: Ahmed Khocht, Yeritxa E. Viera-Negron, Afshin Ameri and Rafik Abdelsayed

Abstract

Background: Chediak-Higashi syndrome (CHS) is a rare autosomal recessive genetic disease. The primary defect is abnormal granule formation in the cells secondary to a mutation of a lysosomal trafficking regulator protein. CHS patients have immune system abnormalities, bleeding abnormalities, and multiple infections including periodontitis. Methods: A 13-year-old African American male presented with severe gingival inflammation, generalized gingival bleeding, and tooth looseness. Comprehensive dental, medical and laboratory evaluations were performed. Results: All teeth exhibited excessive mobility. The gingival tissues were swollen and bled easily. Most sites had probing depth in excess of 10 mm. Dental radiographs showed advanced generalized alveolar bone loss. Areas of skin depigmentation were noted. Blood smear showed presence of intracellular large granules in white blood cells. Platelet function was altered. Gingival histopathology showed an intense chronic inflammatory cell infiltrate and presence of numerous filamentous bacteria. Subgingival microbiological culture showed the presence of Porphyromonas gingvalis, Prevotella intermedia and Tannerella forsythia. Based on the periodontal, medical and laboratory findings a diagnosis of CHS was established. Because of the advanced periodontal condition and the risk of fatal bacterial infections, exodontias were performed. Because of platelet abnormalities the patient developed postoperative bleeding complications that required management with coagulation factor 7. Conclusions: Advanced periodontitis is an important symptom of CHS and may be the first step in the diagnosis of the condition. Due to the weakened immunity of CHS patients, periodontal management is usually unsuccessful. Tooth extractions are recommended to eliminate the periodontal problems and reduce the risk of fatal bacterial infections.

Download Article

Other articles in this issue

Article
Osseous Grafting Part I: Autografts and Allografts for Periodontal Regeneration - A Literature Review Ali Saad AlGhamdi, Othman Shibly and Sebastian G. Ciancio Download
Osseous Grafting Part II: Xenografts and Alloplasts for Periodontal Regeneration - A Literature Review Ali Saad AlGhamdi, Othman Shibly and Sebastian G. Ciancio Download
A Two-Stage Approach Using an Autogenous Masticatory Mucosal Graft and an Autogenous Connective Tissue Graft to Treat Gingival Recession: A Case Report Jun-Beom Park Download
Periodontitis Associated with Chediak-Higashi Syndrome in a Young African American Male Ahmed Khocht, Yeritxa E. Viera-Negron, Afshin Ameri and Rafik Abdelsayed Download
Experimental Gingivitis in Male Khat (Catha edulis) Chewers Nezar Noor Al-hebshi and Mohammed Sultan Al-akā€™hali Download