Papillon-Lefèvre Syndrome

A 30-year-old Puerto Rican woman sought dermatologic attention because of painful feet. Diffuse, hyperkeratotic, fissured plaques with well-defined margins were present on the soles. On one foot, the eruption extended medially to the metatarsophalangeal joint of the great toe; on the other, it involved the lateral aspect of the foot and the Achilles tendon. Mild keratoderma of the palms and psoriasiform patches on the arms were also noted. Oral examination disclosed an edentulous maxilla and only eight teeth in the mandible. Radiographs of the skull showed bilateral calcifications in the falx cerebri, documenting the full-blown Papillon-Lefèvre syndrome. In patients with palmoplantar keratoderma, careful examination of the mouth is essential; if significant dental changes are discovered, roentgenograms of the skull should be obtained.