Papillon-lefe'vre syndrome: neutrophil motility and killing defect in a child with recurrent severe infections

ABSTRACT

A case of palmoplantar hyperkeratosis with periodontosis and a history of recurrent severe pyoderma, pneumonia and multiple liver abscesses is described in a 12 year old girl. The patient demonstrated neutrophil dysfunction characterized by decreased random migration and chemotaxis and defective bactericidal activity. The exact immunopathological mechanism for susceptibility to infections in Papillon-Lefe'vre syndrome patients still remains to be determined. However, the mode of clinical presentation, laboratory findings and response to retinoid treatment, all support the speculation of Papillon-Lefe'vre syndrome as a primary immunologic disease with a variable defect in neutrophil motility and bactericidal activity. The pattern of clinical presentations as skin and periodontal lesions alone or with susceptibility to infection in other sites will change accordingly