Papillon-Lefèvre Syndrome

Abstract

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive condition that causes palmoplantar keratoderma and premature exfoliation of primary and secondary dentition. There are several diseases that can mimic PLS, and it is estimated that the disease is far more prevalent than documented but may either be misdiagnosed or not acknowledged. Radiological exams can provide important clues to differentiate between these syndromes and make an accurate diagnosis.