PAPILLON-LEFEVRE SYNDROME - A REVIEW OF THE LITERATURE AND REPORT OF 4 CASES
PAPILLON-LEFEVRE SYNDROME - A REVIEW OF THE LITERATURE AND REPORT OF 4 CASES
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DOI：
10.1902/jop.1995.66.5.413
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发表时间：
1995-05-01
影响因子：
4.3
通讯作者：
ALUBOSI, MM
中科院分区：
文献类型：
--
作者：
HATTAB, FN;RAWASHDEH, MA;ALUBOSI, MM
本报告描述了4例Papillon-Lefevre综合征（PLS），影响2个约旦家庭，共8名儿童。在A家庭中，6个兄弟姐妹中有3个女孩受到影响;在B家庭中，2个兄弟中的老大。这些患者年龄在4岁半到12岁之间，他们的父母是堂兄弟姐妹，没有受到影响。诊断由病史、临床、实验室和放射学检查确定。所有目前的情况下表现出典型的PLS的临床特征：过度角化的手掌和脚底和存在先进的破坏性牙周炎导致早期损失的乳牙和恒牙列。在病例4中，儿童在6个月大时出现皮肤病变，12岁时出现缺牙。在所有的情况下，有一个关系的严重程度增加的皮肤病变和季节性变化和加剧牙周破坏。恒牙长得早.牙齿无龋，无牙根吸收迹象。牙科医生在PLS患者的诊断和管理中发挥着重要作用。
THIS REPORT DESCRIBES 4 CASES OF PAPILLON-LEFEVRE SYNDROME (PLS) affecting 2 Jordanian families with a total of 8 children. In family A, 3 girls out of 6 siblings were affected; and in family B, the eldest of 2 brothers. The patients were between 4 1/2 and 12 years old, and their parents, who are first cousins, were not affected. Diagnosis was determined by history, and clinical, laboratory, and radiographic examinations. All present cases exhibited the typical clinical features of PLS: hyperkeratosis of the palms and soles and the presence of advanced destructive periodontitis resulting in early loss of both the primary and permanent dentitions. In Case 4, cutaneous lesions were noticed at age 6 months and the child was edentulous by the age of 12 years. In all cases, there was a relationship between increased severity of skin lesions and seasonal variations and intensified periodontal destruction. There was an early eruption of the permanent teeth. The teeth were caries-free with no sign of root resorption. Dentists play a significant role in the diagnosis and management of PLS patients.