Papillon Lefevre syndrome: Bridge between Dermatologist and Dentist

Abstract

Papillon-Lefevre syndrome is a rare autosomal recessively inherited condition which shows features common to both dentistry and dermatology. This disease is characterized by palmoplantar hyperkeratosis and severe periodontitis. Therefore both the dentist and the dermatologist should be aware of the same because an early diagnosis of the syndrome can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Here we present a case report of Papillon-Lefevre syndrome, along with a comprehensive review of the etiology, pathology, clinical features, differential diagnosis and management of the condition. .

Papillon lefevre syndrome- An imperative role of periodontist

“…Yet recent studies have proposed that cathepsin C gene mutation localized to chromosome 11q 14-21 is responsible for the occurrence of this disorder. The expression of cathespin C is known to be evident in the neutrophils and epithelial regions such as soles, palms and keratinised mucosa like gingiva and it is functionally significant in structural growth and development of skin and also in susceptibility to occurrence of periodontal disease (Singla et al 2010, Kaur et al 2013, Kumar et al 2014. Therefore, the characteristic features exemplified in this syndrome include palmarplantar hyperkeratosis, severe loss of periodontium around the teeth and due to the loss of this supporting structure it ultimately results in early loss of dentition.…”

“…Though it shows a negative family history, it can occur in other siblings along with the effected individual (Singla et al 2010). It was first described by two French physicians Papillon and Lefevre and hence the name was proposed accordingly (Singla et al 2010, Kola et al 2014).…”

“…Its prevalence is about 1 to 4 persons per million and shows no sex predilection as well as no racial predominance (Pavankumar et al 2010). However, a genetic predisposition exists with greater chance of occurrence in the consanguineous offspring (Singla et al 2010, Kaur et al 2013). The syndrome is characterised by palmarplantar hyperkeratosis, severe periodontitis, and premature exfoliation of primary and permanent dentition.…”

Case Report of Prosthetic Treatment of a Young Patient Suffering Papilon-Lefever Syndrome

“…Its etiological role hasn't been yet clearly defined and we don't know exactly which immunological, genetic or microbiological factors are effective in this disease [4] [5] [6].…”

“…Using narrow posterior teeth, reduction of vertical height of occlusion, reduction of posterior teeth number and less use of the prosthesis will lead to less loss of the ridge in long terms [4].…”