An unusual presentation of the Papillon-Lefevre syndrome as recurrent liver abscesses.

ABSTRACT

The Papillon-Lefevre syndrome is a rare genetic disorder with a predisposition to severe infections. We describe Papillon-Lefevre syndrome in a 17 year old boy from a family where four out of eight siblings were affected with this disease and who presented with recurrent pyogenic liver abscesses over a period of 9 years, an association never previously reported.