Papillon-Lefèvre syndrome: serum immunoglobulin G (IgG) subclass antibody response to periodontopathic bacteria. A case report.

BACKGROUND: Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder which is characterized by palmar-plantar hyperkeratosis and rapid periodontal destruction of both primary and permanent dentitions. In this case report, we present clinical features, and microbiological and immunological findings of 40 month-old Thai male PLS patient.
METHODS: Microbiological examinations consisted of bacterial culture methods utilizing selective media, morphological identification, and biochemical tests. In addition, the specific serum IgG subclass antibody titers reactive with etiologic periodontal bacteria were determined by the dot-blot immunological analysis and enzyme linked immunosorbent assay (ELISA).
RESULTS: The examinations revealed that the patient harbored 3 major suspected periodontopathic microorganisms, A. actinomycetemcomitans, P. gingivalis, and P. intermedia. The patient's serum IgG1, IgG2, and IgG3, but not IgG4, titers against A. actinomycetemcomitans were dramatically increased. The predominant IgG subclass was IgG1. In contrast, the IgG titers against other tested bacteria, P. gingivalis, P. intermedia, and F. nucleatum, appeared to be similar to those of a healthy control.
CONCLUSIONS: A. actinomycetemcomitans seems to play a pivotal role in the bacteria-host interaction in PLS periodontal pathogenesis. Response of the specific serum IgG subclass antibody titers against the A. actinomycetemcomitans antigen has been demonstrated. This association warrants further investigation.