Rare Diseases Symptoms Automatic Extraction

Evaluation of two siblings with Papillon-Lefèvre syndrome 5 years after treatment of periodontitis in primary and mixed dentition.

[papillon-lefèvre syndrome]

This case report describes the clinical and microbiologic long-term outcome 5 years after periodontal therapy of two siblings diagnosed with Papillon-Lefèvre syndrome (PLS) and tinea capitis.In 2005, two brothers diagnosed with PLS and tinea capitis began periodontal treatment. Both of them showed premature mobility of the primary dentition, markedly increased probing depths, and subgingival Aggregatibacter actinomycetemcomitans (previously Actinobacillus actinomycetemcomitans; Aa). Initial therapy consisted of scaling and root planing based on the concept of full-mouth disinfection, extraction of periodontally hopeless deciduous teeth, and systemic antibiotics. Reevaluation of clinical parameters revealed a dramatic improvement. After that, the patients were enrolled in a stringent maintenance program. Microbiologic monitoring was performed 1 and 5 years after treatment.Five years after initial treatment, the periodontal situation was stable in both patients. Residual deciduous teeth, with the exception of one tooth, could be retained and no further teeth were lost. Further disease progression on the previously involved teeth was controlled, and development of periodontitis on erupting teeth was prevented for a period of 5 years.Even periodontally affected deciduous teeth can be treated successfully in patients with PLS. Suppression of Aa and a stringent maintenance program are of high importance.

Diseases presenting "high importance" symptom

  • papillon-lefèvre syndrome

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