Papillon- Lefevre Syndrome: Report of a case and its management

S Sachdeva,N Kalra,P Kapoor

doi:10.4317/jced.50594

Abstract

Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis. The etiopathogenesis of the syndrome is relatively obscure and immunologic, genetic or possible bacterial etiologies have been proposed. Due to the vast degree of periodontal breakdown involved at such an early age, the dental surgeon is often the first to diagnose the syndrome. This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lefèvre Syndrome. Key words:Papillon-Lefèvre Syndrome, palmoplantar keratoderma, rapidly progressing periodontitis.

Highlights

Papillon-Lefèvre Syndrome (PLS) or keratosis palmoplantaris with periodontopathia is a rare autosomal recessive disorder characterized by diffuse transgradient hyperkeratosis of the palms and soles and severely destructive, rapidly progressive periodontal disease [1]
This paper presents a brief overview of Papillon-Lefèvre syndrome and describes the clinical presentations in a case with typical dental and dermatological findings
The disorder is characterized by diffuse palmoplantar keratoderma and rapidly progressing periodontitis leading to premature loss of both deciduous and permanent teeth

Summary

Introduction

Papillon-Lefèvre Syndrome (PLS) or keratosis palmoplantaris with periodontopathia is a rare autosomal recessive disorder characterized by diffuse transgradient hyperkeratosis of the palms and soles and severely destructive, rapidly progressive periodontal disease [1]. Medical history revealed that the patient had been suffering from recurrent skin infections since an early age with thickening and subsequent peeling of the skin of his hands and feet, for which he had been undergoing intermittent treatment. The Panoramic radiograph demonstrated advanced bone loss giving the teeth a characteristic “Floating in air” appearance (Fig. 2) Routine clinical investigations such as complete blood hemogram, liver profile, renal profile and ultrasound of the abdomen and pelvis were all normal.

Discussion

Conclusion