Papillon‐Lefévre Syndrome: A Review of the Literature and Report of 4 Cases

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Abstract
This report describes 4 cases of Papillon‐Lefévre syndrome (PLS) affecting 2 Jordanian families with a total of 8 children. In family A, 3 girls out of 6 siblings were affected; and in family B, the eldest of 2 brothers. The patients were between 4½ and 12 years old, and their parents, who are first cousins, were not affected. Diagnosis was determined by history, and clinical, laboratory, and radiographie examinations. All present cases exhibited the typical clinical features of PLS: hyperkeratosis of the palms and soles and the presence of advanced destructive periodontitis resulting in early loss of both the primary and permanent dentitions. In Case 4, cutaneous lesions were noticed at age 6 months and the child was edentulous by the age of 12 years. In all cases, there was a relationship between increased severity of skin lesions and seasonal variations and intensified periodontal destruction. There was an early eruption of the permanent teeth. The teeth were caries‐free with no sign of root resorption. Dentists play a significant role in the diagnosis and management of PLS patients. J Periodontol 1995; 66:413–420.