Immunological study of patients with the Papillon-Lefevre syndrome

Papillon-Lefevre syndrome (PLS) is characterized by palmoplantar hyperkeratosis and by premature periodontal breakdown. In view of recent suggestions that this syndrome is associated with increased susceptibility to infections we decided to evaluate several immunological parameters in PLS patients and their healthy siblings and parents. PLS patients, as well as their siblings and parents, had a significantly impaired reactivity to both T and B cell mitogens. This in vitro abnormality was not associated with increased susceptibility to infections. The relationship between the impaired in vitro response to mitogens and the predilection for dermatologic and dental manifestations of PLS is discussed.