Rare Diseases Symptoms Automatic Extraction

Prosthodontic treatment of a patient with Papillon Lefevre syndrome.

[papillon-lefèvre syndrome]

Papillon Lefevre Syndrome is an autosomal recessive disorder. One of the most destructive forms is a periodontal disease. A 19 year female with the syndrome, came for dental treatment. Most of her natural dentition was missing. The remaining five natural teeth had extensive carious and periodontal lesions. Two poor dentures replaced the missing teeth. Her main oral complaints were poor esthetics, teeth sensitiveness, and dentures instability. All the remaining natural teeth were removed and the dentures repaired and rebased in one session. Following wound healing and the maturation of the residual ridge, a set of complete dentures was made. Two years after the delivery of the full dentures, an additional set of dentures were provided for social and emotional reasons. 9 years after the last extractions, the patient refuses to undergo bone augmentation and osseointegrated implant procedures, for future preventive and oral rehabilitation care. There is a strong possibility of chronic bone tissue resorption conducive to dentures instability, mandibular nerves exposures and or, perforation of the maxillary sinuses.