Periodontitis associated with Chediak-Higashi syndrome in a young African American male.
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.
Khocht
Ahmed
A
Department of Periodontology and Oral Implantology, Temple University, Maurice H. Kornberg School of Dentistry, Philadelphia, PA 19140, USA. akhocht@temple.edu
Viera-Negron
Yeritxa E
YE
Ameri
Afshin
A
Abdelsayed
Rafik
R
D000293:Adolescent; D016301:Alveolar Bone Loss / Q000209:etiology; D016866:Bacteroidaceae Infections / Q000150:complications; D001439:Bacteroides / Q000302:isolation & purification; D001442:Bacteroides Infections / Q000150:complications; D002609:Chediak-Higashi Syndrome / Q000150:complications*; D005884:Gingival Hemorrhage / Q000209:etiology; D005891:Gingivitis / Q000209:etiology; D006801:Humans; D008297:Male; D006472:Oral Hemorrhage / Q000209:etiology; D010518:Periodontitis / Q000209:etiology*; D016966:Porphyromonas gingivalis / Q000302:isolation & purification; D019106:Postoperative Hemorrhage / Q000209:etiology; D018713:Prevotella intermedia / Q000302:isolation & purification; D014081:Tooth Extraction / Q000009:adverse effects; D014086:Tooth Mobility / Q000209:etiology