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.