Triangular Lunulae in Papillon-Lefèvre Syndrome_ A Case Report

Background: Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by the presence of palmoplantar hyperkeratosis and severe periodontitis leading to premature loss of dentition.Triangular lunulae is a feature considered pathognomonic for an entirely unrelated condition, the Nail and Patella Syndrome, and has not been previously reported together with Papillon-Lefèvre Syndrome.Case Summary: A 16-year-old girl presenting with a long-term history of palmoplantar hyperkeratosis extensive loss of dentition and a short-term history of pyogenic infection of the right big toe.The examination of the patient revealed a rare finding of triangular lunulae on all fingernails.The patient was diagnosed clinically as a case of Papillon-Lefèvre Syndrome.Evaluation of other family members allowed early diagnosis of the syndrome in another sibling and the timely initiation of treatment to prevent similar morbidity as in the primary patient. Conclusion:Triangular lunulae, considered pathognomonic for Nail-Patella Syndrome, may also be a feature of other syndromes, such as the case here.