Oral management of poorly understood Papillon–Lefèvre syndrome – A case report

Abstract

Rationale: Papillon-Lefèvre syndrome (PLS) is a rare genodermatosis with autosomal-recessive genetic inheritanceand has features mainly palmoplantar hyperkeratosis and rapidly progressing severe periodontitis leading to early loss of deciduous and permanent dentition. Patient Concerns: The patient in the presented report was concerned with loose teeth, gingival bleeding and oral malodour and wanted to get missing teeth replaced. Diagnosis: The diagnosis was made based on medical history, clinical and radiological findings. Interventions: Periodontal phase I therapy included extraction of hopeless teeth, manual and ultrasonic scaling, mechanical and chemical plaque control and periodic maintenance. Missing teeth were restored by removable and fixed prosthesis. Dermatological lesions were conservatively managed. Outcomes: A comprehensive treatment stabilised periodontal disease of the patient and prosthetic rehabilitation improved oral health related quality of life of PLS patient. Lessons: PLS patients require an early diagnosis and a team approach for dermatological, periodontal complications and prosthetic rehabilitation.

Keywords

• cathepsin c
• genodermatosis
• keratoderma
• papillon–lefèvre syndrome
• periodontitis