Papillon-Lefevre Syndrome In An Adolescent Female: A Case Study

摘要

Papillon-Lefevre Syndrome (PLS) is a rare inherited autosomal-recessive condition with one-third of the patients? showing consanguinity of the parents. Lesions are characterised by palmar-plantar hyperkeratosis and hyperhidrosis. Early onset of periodonditis, severe periodontal destruction in both primary and permanent dentitions, and calcification of the duramater form the three important features of this disease. Here, we present a case of a 14-year-old female who presented to the Department of Oral Medicine and Radiology with a complaint of mobility of the teeth since four months. Oral examination of the patient showed generalised mobility of the teeth. General physical examination of the patient showed dry scaly skin on dorsum of bilateral feet, hands, and knee. The patient had familial history positive for consanguinity. The patient was medically diagnosed as positive for PLS. The patients with PLS show combination of dermatological and dental lesions and it requires the dentist to assume a more prominent role in early treatment and rehabilitation. There is a need for symbiotic and synergetic approach between the two specialties for effective management of this rare disease.