The Papillon-Lefèvre syndrome: Keratosis palmoplantaris with periodontopathy
The Papillon-Lefèvre syndrome: Keratosis palmoplantaris with periodontopathy
复制标题
Papillon-Lefèvre 综合征：掌跖角化症伴牙周病
作者：
E. Haneke
乳突-左滴综合征（PLS）是一种常染色体隐性遗传特征，其特征为弥漫性过染性掌足底角化病（PPK）和乳牙和恒牙过早脱落。在大多数情况下，PPK在生命的前3年内被注意到。牙周病变在长出原牙和恒牙后不久就开始了。牙齿受影响的顺序是：牙周组织发炎、牙龈出血、牙袋形成、脱落，最后自发脱落，但没有牙根吸收的明显迹象。在一段无牙期后，在长出第二齿后不久，同样的过程又重新开始。异位颅内钙化、智力低下和对感染的易感性增加经常出现在PLS患者中，因此可能被视为兼性体征。
SummaryThe Papillon-Lefèvre syndrome (PLS) is an autosomal recessive trait characterized by diffuse transgredient palmar-plantar keratosis (PPK) and premature loss of both the deciduous and permanent teeth. In most cases, the PPK is noted within the first 3 years of life. The periodontal lesions begin shortly after the start of both the primary and the permanent dentitions. The teeth are affected in the order of their eruption, exhibiting inflammation of the periodontal tissue, bleeding of the gums, pocket formation, lossening, and finally spontaneous exfoliation without showing definite signs of root resorption. After an edentulous interval, the same process begins anew shortly after the second dentition.Ectopic intracranial calcifications, mental retardation, and increased susceptibility to infections have often been seen in PLS patients and may thus be regarded as facultative signs.