Abstract
Background: Papillon-Levefre Syndrome (PLS) is a rare genetic disorder with prevalence of 1-4 cases per millions of birth, characterized by the presence of palmar-plantar hyperkeratosis and rapid periodontal destruction of both the primary and secondary dentition. Purpose: This clinical report described such a rare condition with special attention on its diagnostic characterization, various treatment options and Prosthodontic rehabilitation. Case: Five years-old boy accompanied by his mother came to Pediatric Dentistry Specialist Clinic, Universitas Airlangga Dental Hospital - Surabaya with the chief complaint of gradually loss of upper and lower teeth since the patient was 3 years old. Case Management: Removable partial denture was chosen because it was considered as an appliance that can recover the function of mastication, speech and aesthetic patient. Removable partial denture was designed to replace 51, 52, 53, 54, 55, 61, 62, 63, 64, 65, 72, 73, 74, 75, 82, 83, 84 and 85 with retention on 16, 26, 36 and 46, and anterior bite riser on 31 and 41. Conclusion.Removable partial denture is the treatment of choice for children with PLS who are still in the growth period because it can recover mastication and aesthetic functions, have a good aesthetic, and easy maintenance that could done by the child.
Highlights
Papillon-Levefre Syndrome (PLS) is a rare autosomal recessive genetic disorder with a prevalence of [1,2,3,4] cases per million of births
Oral manifestation is aggressive periodontitis leading to premature loss of deciduous and permanent teeth at the very young age.[1]
Removable partial denture was chosen because it was considered as an appliance that can recover the function of mastication, speech and aesthetic patient
Summary
Papillon-Levefre Syndrome (PLS) is a rare autosomal recessive genetic disorder with a prevalence of [1,2,3,4] cases per million of births. Oral manifestation is aggressive periodontitis leading to premature loss of deciduous and permanent teeth at the very young age.[1] The development and eruption of primary dentition is frequently associated with gingivitis and periodontitis. The patient consults dentist first because of premature teeth loss and associated problems. This clinical report described such a rare condition with special attention on its diagnostic characterization, various treatment options and Prosthodontic rehabilitation. Removable partial denture was chosen because it was considered as an appliance that can recover the function of mastication, speech and aesthetic patient. First control (five months after insertion) patient came with good general condition, the removable partial denture rarely used because its discomfort in speech and soreness. The patient feels more comfortable with the new appliance (Figure 8) Patient and parent are again given education of how to use and care of removable partial denture appliances as well as instructions to maintain oral hygiene
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