Papillon-Lefevre syndrome with arachnodactyly and associated aggressive periodontitis

Abstract

Papillon-Lefevre Syndrome (PLS) is a autosomal recessive genetic disorder. The prevalence of this disorder has been reported to be 1 to 4 per million in the general population. The prime features of PLS include palmar-plantar hyperkeratosis along with premature loss of deciduous and permanent teeth due to aggressive periodontitis. Radiological feature involves severe destruction of alveolar bone giving teeth a typical “floating in air” appearance along with intra-cranial calcifications. Clinical findings of disease specific dermatologic manifestations and periodontal involvement forms the framework of the diagnosis. Clinical significance: As PLS involves severe irreparable destruction of alveolar bone and tooth loss, both dental clinicians and dermatologists opinion is required for both initial and long term management of this disease followed by psychological counselling.

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

“…Periodontal inflammation plays a dominant role in clinical diagnosis( Henderson et al, 2009 ). Because the periodontium is involved, the dentist is typically the first to diagnose the PLS( Tariq et al, 2022 ). One prior study showed that patients with PLS exhibit similar bacterial flora, including spirochetes, and populations of Epstein–Barr virus and cytomegaloviruses, as in individuals with chronic periodontitis.…”

“…One prior study recommended antibiotics such as amoxicillin or amoxicillin with clavulanic acid. The suggested dosage was 20–50 mg/kg/day or 20–40 mg/kg/day, respectively, administered in divided doses every 8 h. It is advised to administer antibiotics for a duration of two weeks following tooth extraction to prevent postoperative complications( Tariq et al, 2022 ). In the book, Pediatric Dentistry Infancy through Adolescence, successful treatment outcomes in children were also documented through the implementation of antibiotic therapy( Casamassimo et al, 2012 ).…”

“…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 ).…”