Levo Y, Wollner S, Hacham-Zadeh S
Clin Exp Immunol. 1980 May;40(2):407-10.
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.
掌跖角化牙周破坏综合征（PLS）的特征为掌跖过度角化和过早的牙周组织破坏。鉴于最近有观点认为该综合征与感染易感性增加有关，我们决定评估PLS患者及其健康的兄弟姐妹和父母的多项免疫参数。PLS患者及其兄弟姐妹和父母对T细胞和B细胞有丝分裂原的反应性均显著受损。这种体外异常与感染易感性增加无关。本文讨论了对有丝分裂原体外反应受损与PLS皮肤和牙齿表现倾向之间的关系。
