- link with syndromes.
11.12 PERIODONTITIS AS A MANIFESTATION OF SYSTEMIC DISEASE
11.12.0 Introduction
The genetic basis for aggressive periodontitis in particular is substantiated by the
definite association between the condition and a number of rare inherited medical
conditions and syndromes (637HTable 11.1). The pattern of inheritance reflects a single
gene disorder, commonly involving inherited defects of neutrophils, enzyme
reactions, or collagen synthesis.
11.12.1 Papillon-Lefevre syndrome (PLS)
This syndrome is characterized by palmar-plantar hyperkeratosis, premature loss of
primary and permanent dentitions, and ectopic calcifications of the falx cerebri. Some
patients show an increased susceptibility to infection. The syndrome is an autosomal-
recessive trait with a prevalence of about 1-4 per million of the population.
Consanguinity of parents is evident in about one-third of cases.
Rapid and progressive periodontal destruction affects the primary dentition with an
onset at about 2 years (638HFig. 11.15). Exfoliation of all primary teeth is usual before the
permanent successors erupt and patients may be edentulous by the mid to late teens.
Cases of a late-onset variant of PLS have also been described in which the palmar-
plantar and periodontal lesions are relatively mild and only become evident in the
permanent dentition. An extensive family dental history supported by clinical,
laboratory, and radiographic examinations confirms the diagnosis.
639H
Fig. 11.15 Papillon-Lefevre syndrome in
a 3-year-old boy. (a) Radiographic
appearance showing almost total bone
loss around maxillary anterior teeth. (b)
Hyperkeratosis of the palms of the hand.