Papillon-Lefèvre-Syndrom

Abstract

Papillon-Lefèvre syndrome in a 13-year-old boy is described. His two great-grandfathers were first-degree cousins (consanguinity). However, no other case of the syndrome nor of palmoplantar keratoderma exists in the family. The palmoplantar keratoderma, which had started before becoming 1 year of age, is usually more pronounced during spring and autumn as well as during feverish diseases. However, it is of the trans-gradient type and is not necessarily very severe. The loss of the deciduous and permanent teeth was a consequence of severe juvenile parodontitis and of bone destruction. The boy could extract his teeth easily by himself and has total prothesis since the age of 14 years. Every treatment was unsuccessful.

Papillon-Lefèvre Syndrome

“…Papillon-Lefèvre Syndrome cases; if we add the case recently reported by Krebs [10] and the present case, there are 13 descriptions which include a total of 16 pa tients, of whom 12 are males and 4 females. Even though the literature generally indi cates an equality of sex distribution, we see in these circumstances the masculine pre dominance to be quite significant.…”

Periodontal treatment of patients with Papillon–Lefèvre syndrome: a 3‐year follow‐up

“…Early case reports on periodontal treatment in PLS patients describe unsuccessful outcome and tooth loss leading to edentulism as an unavoidable part of this syndrome (Gorlin et al 1964, Haim & Munk 1965, Krebs 1978). Treatment with oral hygiene instructions and scaling and root planing has been reported unsuccessful (Hathway 1982, Bimstein et al 1990, Hattab et al 1995).…”

Manifestation of heterozygosity in Papillon‐Lefèvre syndrome?

“…None of his 7 sibs exhibits similar symptoms, nor do 7 children of 4 of these sibs. In the literature, several patients with one parent losing teeth early in life have been recorded [Krebs, 1978;Munford, 1976;Pfister, 19651. Unfortunately, details are not published.…”