Open AccessPapillon-Lefevre syndrome with arachnodactyly and associated aggressive periodontitis
Author(s) -
Mehak Tariq,
Munaf Maknojia,
Sandeep Saurabh,
Amarnath,
Shubhangi Rajbhoj,
Sugandha Arya
Publication year - 2022
Publication title -
international journal of health sciences (ijhs) (en línea)
Language(s) - English
Resource type - Journals
eISSN - 2550-6978
pISSN - 2550-696X
DOI - 10.53730/ijhs.v6ns3.5913
Subject(s) - medicine , dental alveolus , periodontitis , aggressive periodontitis , dentistry , deciduous teeth , population , dermatology , environmental health
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.