Papillon - Lefevre Syndrome - A Brief Review of Diagnosis &amp; Management

Kumar, Dr Tarun; Pillai, Dr Ajay Kumar; Kulkarni, Dr Parimala; Moghe, Dr Swapnil; Vishnu, Dr Vineesh; Yadav, Dr Saurabh Dhanraj

doi:10.9790/0853-131169295

Cited by 4 publications

(2 citation statements)

References 8 publications

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“…Second, some pathogenic microorganisms like Capnocytophaga gingivalis, Porphyromonas gingivalis, Actinobacillus actinomycetemcomitans, Peptostreptococcus micros, Fusobacterium nucleatum, and spirochetes have been concerned as the causal agents for periodontal problems in PLS. 2 7 16 …”

Section: Discussionmentioning

confidence: 99%

Papillon–Lefèvre Syndrome: A Rare Case Report of Two Brothers and Review of the Literature

Fageeh

2018

International Journal of Clinical Pediatric Dentistry

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Papillon-Lefèvre is an autosomal recessive syndrome that starts in early periods of childhood. Characteristic features include palmar plantar hyperkeratosis, aggressive periodontal disease, and a tendency for dry and chopped skin, thin and sparse hair. Patients show signs of premature tooth loss at the age of 2 to 4 years, which is then followed by the loss of permanent dentition during adolescence. The presence of both skin and oral lesions in this syndrome differentiates this unusual genodermatosis from other pathology of palmoplantar keratoderma (PPK). The etiopathogenesis of this syndrome is somewhat obscure; however, immunologic, genetic, and possible bacterial etiologies have been proposed. The dental practitioner is often the first to diagnose the disease, as there is a significant degree of periodontal breakdown that is involved at an early age. This report presents a clinical presentation of two brothers detected with Papillon-Lefèvre syndrome (PLS).How to cite this article: Fageeh HN. Papillon-Lefèvre Syndrome: A Rare Case Report of Two Brothers and Review of the Literature. Int J Clin Pediatr Dent 2018;11(4):352-355.

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Section: Discussionmentioning

confidence: 99%

Papillon–Lefèvre Syndrome: A Rare Case Report of Two Brothers and Review of the Literature

Fageeh

2018

International Journal of Clinical Pediatric Dentistry

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“…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.…”

Section: Discussionmentioning

confidence: 99%

Papillon lefevre syndrome- An imperative role of periodontist

Kumari

Kumar

priya

et al. 2016

IJDR

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Papillon Lefevre syndrome (PLS) is a rarely encountered disorder that shows autosomal recessive inheritance. Clinically, it exhibits hyperkeratotic plaques on palmar plantar surfaces, severe periodontal loss and precocious shedding of primary as well as permanent dentition. The reason for this exceptional clinical presentation is vague and speculative. We are reporting one such case of a 23year old female patient.

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