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 decide to evaluate several immunological parameters in PLS patients an...

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Veröffentlicht in:Clinical and experimental immunology 1980-05, Vol.40 (2), p.407-410
Hauptverfasser: Levo, Y, Wollner, S, Hacham-Zadeh, S
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creator Levo, Y
Wollner, S
Hacham-Zadeh, S
description 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 decide 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.
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subjects Adolescent
Adult
B-Lymphocytes - immunology
Child
Female
Humans
Immunoglobulins - analysis
Keratoderma, Palmoplantar - immunology
Male
Mitogens - pharmacology
Papillon-Lefevre Disease - genetics
Papillon-Lefevre Disease - immunology
Rosette Formation
T-Lymphocytes - immunology
title Immunological study of patients with the Papillon--Lefevre syndrome
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