IMMUNOLOGICAL STUDY OF PATIENTS WITH THE PAPILLON-LEFEVRE SYNDROME

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  • 1 January 1980
    • journal article
    • research article
    • Vol. 40  (2) , 407-410
Abstract
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, several immunological parameters were evaluated in PLS patients and their healthy siblings and parents. PLS patients and their siblings and parents had a significantly impaired reactivity to both T [thymus-derived] and B [bone marrow-derived] 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.