Papillon-Lefèvre syndrome: Case report of 3 siblings in consanguineous family

Ayesha Anwar, Wasifa Hayat, Shahbaz Aman


The Papillon-Lefèvre syndrome (PLS) is a very rare syndrome of autosomal recessive inheritance characterized by palmoplantar hyperkeratosis and early onset periodontitis, leading to premature loss of both primary and permanent dentition. We report three cases of PLS in the same family who presented with all characteristic features. All patients presented with persistent thickening, flaking and scaling of the skin of palms and soles, mobility and rapid loss of teeth. Severe gingival inflammation, abscess formation, and deep periodontal pockets along with loss of teeth were evident on intraoral examination.



Palmoplantar hyperkeratosis, Papillon-Lefèvre syndrome, periodontitis

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Papillon MM, Lefèvre P. Two cases of symmetrical, familial (Meleda's malady) palmar and plantar keratosis of brother and sister: Coexistence in two cases with serious dental changes. Bull Soc Fr Dermatol Syphiligr. 1924;31:82–7.

Muppa R, Prameela B, Duddu M, Dandempally A. Papillon-lefevre syndrome: a combined approach from the dermatologist and dentist—a clinical presentation. Indian Journal of Dermatology. 2011;56(6):740–743.

Nakajima K, Nakano H, Takiyoshi N et al. Papillon-Lefèvre syndrome and malignant melanoma. A high incidence of melanoma development in Japanese palmoplantar keratoderma patients. Dermatology. 2008;217(1): 58–62.

Gorlin RJ. Of palms, soles, and gums. J Med Genet. 2000;37:81–2.

Posteraro AF. Papillon-Lefèvre syndrome. J Ala Dent Assoc. 1992;76:16–9.

Khandpur S, Reddy BS. Papillon-Lefèvre syndrome with pyogenic hepatic abscess: A rare association. PediatrDermatol. 2001;18:45–7.

Almuneef M, Al Khenaizan S, Al Ajaji S, Al-Anazi A. Pyogenic liver abscess and Papillon-Lefèvre syndrome: Not a rare association.Pediatrics. 2003;111:85–8.

Sreeramulu B, Shyam ND, Ajay P, Suman P. Papillon–Lefèvre syndrome: clinical presentation and management options. Clin Cosmet Investig Dent. 2015;7:75–81.

Lundgren T, Renvert S. Periodontal treatment of patients with Papillon-Lefèvre syndrome: A 3-year follow-up. J Clin Periodontol. 2004;31:933–8.

Shah AF, Tangade P, Agarwal S. Papillon-Lefevre syndrome: reporting consanguinity as a risk factor. Saudi Dental Journal. 2014;26(3):126–131.

Bhavsar MV, Brahmbhatt NA, Sahayata VN, Bhavsar NV. Papillon-Lefevre syndrome: case series and review of literature. J Indian Soc Periodontol. 2013;17(6):806–811.

Biraggari SK, Reddy KKM, Sudhakar J, Bugude SS, Nichenametla R, Hakeem MA et al. Papillion-Lefèvre Syndrome:Periodontists Perspective. Case Rep Dent. 2015; 2015:137381.

Ashri NY. Early diagnosis and treatment options for the periodontal problems in Papillon-Lefèvre syndrome: A literature review. J Int Acad Periodontol. 2008;10: 81–6.

Nickles K, Schacher, Schuster G, Valesky E, Eickholz P. Evaluation of two siblings with Papillon-Lefèvre syndrome 5 years after treatment of periodontitis in primary and mixed

dentition. J Periodontol. 2011;82(11):1536–1547.

Sharma A, Kaur G, Sharma A. Papillon-Lefevre syndrome: A case report of 2 affected siblings. J Indian Soc Periodontol. 2013;17(3): 373–377.

KhanFY, Jan SM, Mushtaq M.Papillon-Lefèvre syndrome: Case report and review of the literature. J Indian Soc Periodontol. 2012;16(2): 261–265.


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