基底细胞痣综合征的诊治  

The diagnosis and treatment of basal cell naevus syndrome

作　　者：王寅[1,2] 郭晶洁[1] 杨钧[1] 孙庚林[1] 李龙江[2] 

机构地区：[1]天津医科大学总医院口腔颌面外科,天津300052 [2]四川大学华西口腔医院头颈肿瘤外科,四川成都610041

出　　处：《国际口腔医学杂志》2009年第3期260-262,共3页International Journal of Stomatology

摘　　要：目的研究基底细胞痣综合征的发病情况、临床表现、诊断和治疗方法。方法回顾性总结1999—2007年期间在天津医科大学总医院治疗的4例基底细胞痣综合征的诊断和治疗过程。结果该综合征患者具有典型的面部特征,皮肤可见基底细胞痣(癌),手掌或足底可见特殊凹陷,骨骼系统常有单侧或双侧肋骨分叉和异常钙化,大部分患者有多发性颌骨角化囊肿的口腔表现。本组病例未发现家族史。结论多发性颌骨角化囊肿是基底细胞痣综合征的重要临床表现之一,及时正确的治疗能使患者获得良好的生存质量。Objective The purpose of this study was to evaluate the clinical manifestation, diagnosis and treatment of basal cell naevus syndrome （BCNS）. Methods Reviewed the procedure of diagnosis and treatment by analyzing the records of 4 inpatients of BCNS in General Hospital Affiliated to Tianjin Medical University from 1999 to 2007. Results The inpatients characterized with typical facial features, cutaneous basal cell naevus （carcinoma） were obviously seen on the skin, palmar or plantar pits were viewed, bilamellar calcification of falx cerebri, bifid, fused or markedly splayed ribs were found, and histologically confirmed odontogenic keratocysts of jaw could be detected in most cases of BCNS. No family history was found in all cases of the group. Conclusion Multiple odontogenic keratocysts of jaw is one of the most important clinical manifestation of BCNS. The correct and prompt treatment will provide the patients with better life quality.

关 键 词：基底细胞痣综合征 颌骨角化囊肿 诊断 治疗 

分 类 号：R758.51[医药卫生—皮肤病学与性病学]