title: Treatment of the periodontal component of Papillon-Lefèvre syndrome.
abstract: A 9-year-old girl was treated for the periodontal component of Papillon-Lefèvre syndrome, an autosomal recessive disease characterized by palmarplantar hyperkeratosis and premature loss of teeth. Initially, the patient was found to have a polymorphonuclear leukocyte chemotactic dysfunction, defective leukocyte adherence, and deep periodontal pockets harboring presumptive periodontopathic bacteria. After unsuccessful treatment with combined mechanical therapy and 2 different antibiotics, all of the patient's erupted teeth were extracted in an attempt to minimize the chance of infection of teeth yet to erupt. At age 16 years, the now-erupted teeth have normal gingiva and crevice depths, radiographs show no evidence of periodontal pathology, no periodontopathic bacteria are detected in gingival crevices, and leukocyte function is normal.
mesh_terms: D001419:Bacteria / Q000302:isolation & purification; D002648:Child; D003773:Dental Plaque / Q000382:microbiology; D004917:Erythromycin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000503:physiopathology; D010214:Papillon-Lefevre Disease / Q000503:physiopathology; D010510:Periodontal Diseases / Q000188:drug therapy / Q000382:microbiology / Q000628:therapy*; D013752:Tetracycline / Q000627:therapeutic use; D014081:Tooth Extraction
chemical_list: D004917:Erythromycin; D013752:Tetracycline