سندروم پاپیلون لفور در یک کودک 4 ساله: گزارش یک مورد بیماری نادر و پیگیری سه ساله آن

Papillon-Lefèvre syndrome is an extremely rare autosomal recessive condition. It is characterized by severe destruction of the periodontium and hyperkeratotic skin lesions on palms, soles, knees, elbows and in some cases calcification of the Dura. The features usually appear together between the ages of 2 and 4 years. Severe periodontal destruction leads to the early shedding of primary and permanent dentition. Case presentation: A 4-year-old male child was referred to our private clinic with a chief complaint of pain and mobility of the teeth and difficulty in mastication. He had severe mobility in molar teeth. Erythematous keratotic plaques in palms and soles were revealed. According to clinical, radiographic features and pathologic findings, the patient was diagnosed as Papillon-Lefèvre syndrome occurring for the first time in Zanjan. He received periodontal and dermatological treatments. Conclusion: Papillon-Lefèvre syndrome affects the quality of life at a very young age. Since the patient features are common to periodontics and dermatologists, full awareness of how to diagnose and manage these feature seems crucial for both fields. Early diagnosis of PLS improves prognosis and quality of life in these patients.   Keywords: Papillon-Lefèvre syndrome, Palmoplantar keratoderma, Periodontitis