Papillon–Lefevre Syndrome: A Case Report with Review of Literature
Papillon–Lefevre Syndrome(PLS) is a very rare syndrome of autosomal recessive inheritance characterised by palmar plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. The palmar plantar keratoderma typically has its onset between the ages of 1 and 4 years and severe periodontitis starts at the age of 3 or 4 years. The exact pathogenesis of these clinical events remains mainly speculative. An early diagnosis of the syndrome can help preserve the teeth by early institution of treatment, using a multidisciplinary approach. We report a case of 17-year- old female patient withPLS.
Janjua SA, Khachemoune A. Papillon–Lefevresyndrome: a case report with review of literature. Dermatol Online
J. (2004). 10: 13.
Hart TC, Shapira L. Papillon–Lefevresyndrome. Periodontol. (1994). 6: 88–100. 3. Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmar–plantar hyperkeratosis and premature periodontal destruction of the teeth. J ediatr. (1964). 65: 895–908.
Shafer, Hine, Levy. Diseases of Periodontium. A textbook of oral pathology. 4th ed. WB Saunders
Company: Philadelphia. (1993). 797–8.
Reyes VO, King Ismael D, Abad Venida L. Papillon–Lefevresyndrome. Int J Dermatol. (1998). 37: 268–70.
Hattab FN, Rawashdeh MA, Yassin OM, al-Momani, al-Ubosi MM. Papillon–Lefevre Syndrome: a review
of literature and report of 4 cases. J Periodontal. (1995). 66: 413–20.
Shah J, Goel S. Papillon–Lefevresyndrome: two case reports. Indian J Dental Res. (2007). 18: 210–3.
Ulbro C, Crossner CG, Nederfors T, Alfadely A. Dermatologic and oral fi ndings in a cohort of 47 patients
with Papillon–Lefevresyndrome. J Am Acad Dermatol. (2003). 48: 345–51.
Almuneef M, Al Khenaizan S, Al Ajaji S, Al Anazi A. Pyogenic liver abscess and Papillon–Lefevre Syndrome:
not a rare association. Pediatrics. (2003). 111: e85–8.
Ghaffar KA, Zaharan FM, Fahmy HM, Brown RS. Papillon–Lefevre Syndrome: neutrophil functioning of
cases from 4 families in Egypt. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. (1999). 88: 320–5.
Hart TC, Hart PS, Bowden DW, Michalec MD, Callison SA, Walker SJ,et al. Mutations of the cathepsin C gene
areresponsible for Papillon–Lefevresyndrome. J Med Genet. (1999). 36: 881–7.
Angel TA, Hsu S, Kornbleuth SI, Kornbleuth J, Kramer Em. Papillon–Lefevre Syndrome: a case report of four
siblings. J Am Acad Dermatol. (2002). 46: s8–10.