Abstract
Introduction: Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disorder of keratinisation, characterized by palmoplantar hyperkeratosis, periodontitis and early loss of dentition. Since these features are common to both dentistry and dermatology, members of both disciplines should be aware of the same because an early diagnosis of this condition can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Case Series: The authors here present two cases of Papillon-Lefevre syndrome in siblings, having all of the characteristic features, along with a comprehensive review of the etiology, pathology, clinical features, differential diagnosis and management of the condition. Conclusion: The main priority of physician's strategy is to refer the PLS patients at the earliest to a periodontist for the periodontal management of permanent dentition. Based on the previously reported series and our clinical experience, non-surgical periodontal therapy yielded better results when started immediately after the extraction of deciduous dentition.
Highlights
PapillonLefevre Syndrome (PLS) is a rare autosomal recessive disorder of keratinisation, characterized by palmoplantar hyperkeratosis, periodontitis and early loss of dentition
Periodontitis, the second most common feature of PLS starts at age of three or four years [5]
Papillon Lefevre syndrome can drastically affect the psychology of children because of social and esthetical problems
Summary
Papillon Lefevre syndrome (PLS), known as palmoplantar keratoderma with periodontitis, is an inherited disorder of keratinisation. The dental panoramic radiograph showed severe alveolar bone loss in relation to the existing permanent teeth up to the apical third of the roots, giving the teeth a ‘floating in air’ appearance (Figure 3). But not a well formed keratotic plaques on the skin of the palms and soles The extension of these plaques to the dorsal surface of the finger joints and over the dorsal surfaces of the feet was mild and not as marked as in case 1 (Figure 4). On intra oral examination, it was found that she was wearing a complete denture set for ten years All her permanent tooth were lost at the age of 15, except her lower third molar, which persisted with marked inflammation around the tooth (Figure 5). The lateral view of the skull radiograph showed no evidence of intracranial calcification
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