Use of Removable Partial Denture in Child with Papillon-Lefevre Syndrome: Case Report

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.

Papillon-Lefèvre syndrome in dental pediatric patient: A comprehensive review

“…The oral manifestation in PLS is the Aggressive of periodontal disease, characterized by early and extensive tooth loss, representing a key feature of PLS( Hart & Shapira, 1994 ). Premature exfoliation of primary teeth in patients with PLS typically occurs around the age of 4–5 years, while premature loss of the permanent teeth is commonly observed between the ages of 13–15 years( Saskianti et al, 2020 ). In infants and young children affected by PLS, the oral mucosal and gingival tissues initially appear normal.…”

“…Due to early severe alveolar bone resorption, prosthetic management presents a notable challenge in these patients. Certain studies have proposed an alternative approach( Saskianti et al, 2020 ) involving the extraction of all primary teeth at least six months before the eruption of the first permanent tooth( Tariq et al, 2022 ) as a means to preserve alveolar bone height and facilitate subsequent prosthodontic rehabilitation. This may include the utilization of osseo-integrated implants to address the functional and aesthetic needs of these patients( Etöz et al, 2010 ).…”

“…The comprehensive care of patients with PLS requires a multidisciplinary strategy combining teams of pediatricians, dermatologists, and pediatric dentists. In the treating children with PLS who are still growing, removable partial dentures are the best option, as they allow for easy maintenance, good aesthetics, and the recovery of mastication and aesthetic functions( Saskianti et al, 2020 ). A systematic review provided evidence indicating that dental implants exhibit ankylosed teeth and are contraindicated in teenagers and individuals who are still in the growth phase due to the potential risk of implant infrapositioning( Atarbashi-Moghadam et al, 2020 ).…”

“…Papillon – Lefèvre syndrome (PLS), initially described by French physicians Papillon and Lefèvre( Muppa et al, 2011 ), is a rare genetic condition characterized by widespread palmoplantar hyperkeratosis, which affects the skin on the palms of the hands and soles of the feet, with severe periodontal inflammation that can result in the early loss of primary and permanent teeth. PLS follows an autosomal recessive pattern of inheritance( Saskianti et al, 2020 ), and is classified as a form of genetically and clinically diverse palmoplantar keratodermas or keratoses (PPKs). These conditions share a common characteristic of hyperkeratotic lesions primarily affecting the palms and soles.…”