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- DOI 10.18231/j.jds.10033.1485746081
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Papillon Lefevre syndrome-A series of two cases involving siblings with a review of literature
Papillon-Lefevre syndrome (PLS), a rare autosomal recessive disorder, was initially elucidated by Papillon and Lefevre in 1924. It is classically characterized by a combination of cutaneous and oral anomalies; cuateneous manifestations like gradual palmo-plantar keratosis and oral changes like variably progressive periodontitis, involving both deciduous and permanent dentitions are the hallmarks. Also, neurologic manifestations like falx cerebri calcifications and mental retardation occasionally have been reported. Mutation of Cathepsin C gene is held responsible for PLS. The dermatologic lesions are managed with retinoids, along with corticosteroids and/or topical antibiotics. The oral lesions are controlled thorough optimal oral hygiene measures, prophylaxis, antibiotic usage, and extraction of the affected mobile teeth in severe instances. Herein, we describe a case series comprising of two cases of PLS involving siblings- a brother and an elder sister, with an emphasis regarding their relevant diagnostic aspects.
References
- Neville BW, Damn DD, Allen CM, Chi AC. Periodontal Diseases. Oral and Maxillofacial Pathology. 5th Ed. St Louis, Elsevier; 2024:167–70.
- Zhao Yu-Lei, Feng Zhao, Hu Bin, Chen Liu-Qing. Papillon-Lefèvre Syndrome: A Case Report. Int J Dermatol Venereol. 2021;4(2):97–
- Giannetti L, Apponi R, Dello Diago AM, Jafferany M, Goldust M, Sadoughifar R. Papillon-Lefèvre syndrome: Oral aspects and treatment. Dermatol Ther. 2020;33(3):e13336.
- 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):352–5.
- Raja Rajeswari K, Almansour R, Alrajhi F, Fahad Binmeqren A, Shayan Albaqami M, Abdullah Albarrak R. Papillon-Lefèvre syndrome in dental pediatric patient: A comprehensive review. Saudi Dent J. 2024;36(5):682–7.
- Kola MZ. Prosthodontic management of Papillon–Lefèvre syndrome with special focus on its characterization and diagnosis: a clinical report. J Adv Med Dent Sci Res. 2014;2(4):97–104.
- Upadhyaya JD, Pfundheller D, Islam MN, Bhattacharyya I. Papillon-Lefèvre syndrome: A series of three cases in the same family and a literature review. Quintessence Int. 2017;48(9):695–
- Sachdeva S, Kalra N, Kapoor P. Papillon–Lefèvre syndrome: report of a case and its management. J Clin Exp Dent. 2012;4(1):e77–e81
- Mangal S, Ganapathy D. Awareness of Papillon-Lefevre Syndrome among dental students. J Surv Fisheries Sci. 2023;10(1S):3891–9.
- Ketterer S, Gomez-Auli A, Hillebrand LE, Petrera A, Ketscher A, Reinheckel T. Inherited diseases caused by mutations in cathepsin protease genes. FEBS J. 2017;284(10):1437–54.
- Castori M, Madonna S, Giannetti L. Novel CTSC mutations in a patient with Papillon–Lefèvre syndrome with recurrent pyoderma and minimal oral and palmoplantar involvement. Br J Dermatol. 2009;160(4):881–3.
- Adamski Z, Burchardt D, Pawlaczyk-Kamienska T, Borysewicz- Lewicka M, Wyganowska-Swiątkowska M. Diagnosis of Papillon- Lefevre syndrome: Review of the literature and a case report. Adv. Dermatol Allergol. 2020;37(5):671–6.
- Albandar JM, Khattab R, Monem F, Barbuto SM, Paster BJ. The subgingival microbiota of Papillon-Lefevre syndrome. J Periodontol. 2012;83(7):902–8
- Sreeramulu B, Haragopal S, Shalini K, Sudha MD, Kiran G. The prosthodontic management of a young edentulous patient with the papillon lefevre syndrome-A rare case report. J Clin Diagn Res. 2012;6(10):1808–11.
- Tariq M, Maknojia M, Saurabh S, Amarnath Rajbhoj S, Arya S. Papillon-Lefevre syndrome with arachnodactyly and associated aggressive periodontitis. Int J Health Sci. 2022;6(S3):1984–94.
How to Cite This Article
Vancouver
Mahato B, Sarangi S, Ghosh A, Mazumdar A. Papillon Lefevre syndrome-A series of two cases involving siblings with a review of literature [Internet]. J Dent Spec. 2025 [cited 2025 Oct 01];13(2):295-298. Available from: https://doi.org/10.18231/j.jds.10033.1485746081
APA
Mahato, B., Sarangi, S., Ghosh, A., Mazumdar, A. (2025). Papillon Lefevre syndrome-A series of two cases involving siblings with a review of literature. J Dent Spec, 13(2), 295-298. https://doi.org/10.18231/j.jds.10033.1485746081
MLA
Mahato, Basudev, Sarangi, Snehanjan, Ghosh, Abhishek, Mazumdar, Anjana. "Papillon Lefevre syndrome-A series of two cases involving siblings with a review of literature." J Dent Spec, vol. 13, no. 2, 2025, pp. 295-298. https://doi.org/10.18231/j.jds.10033.1485746081
Chicago
Mahato, B., Sarangi, S., Ghosh, A., Mazumdar, A.. "Papillon Lefevre syndrome-A series of two cases involving siblings with a review of literature." J Dent Spec 13, no. 2 (2025): 295-298. https://doi.org/10.18231/j.jds.10033.1485746081