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硬皮病与CREST综合征:牙科病例报告

Scleroderma and CREST syndrome: a case report in dentistry.

作者信息

Lauritano D, Bussolati A, Baldoni M, Leonida A

机构信息

Dental Clinic Neuroscience, Milano-Bicocca University, Monza, Monza e Brianza, Italy.

出版信息

Minerva Stomatol. 2011 Sep;60(9):443-65.

Abstract

CREST syndrome is part of the heterogeneous scleroderma group of autoimmune diseases that cause thickening, hardening and tightening of the connective tissue in different parts of the body, and it may lead to complex disorders. CREST syndrome is characterized by the coexistence of calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactily and telangectasia. A 72-year-old caucasian woman is referred to the S. Gerardo Hospital of Monza, with a chief complaint of oral pain and difficulties in deglutition and eating, associated with denture instability and difficulties to fit it. She had been previously diagnosed with Raynaud's phenomenon, and afterwards with CREST syndrome. Extra-oral examination underlined taut, thickened and rigid skin, pallid-red irregular maculae all over the face, telangiectasias and acrocyanosis. Intra-oral examination showed no alteration of the mucosa, but we can observe tongue rigidity and some speckled red alternating with white spots on the hard palate and in the vestibule. We undermitted the patient the dental treatment of Sjogren's syndrome. The management of the Sjogren's syndrome is symptomatic and empirical, and involves the use of saliva secretion stimulators, salivary substitutes and coadjuvants. Dental treatment and prophylaxis are important to prevent the consequences of xerostomia, such as rampant caries, based on the administration of topical fluoride in toothpastes and rinses, and supplemented by fluoride gels and varnishes. Instruction and reinforcement of oral hygiene, along with frequent dental assessment and management by the dentist are essential measures to preserve the oral health of those affected with CREST syndrome in progression to SS, complicated with Sjogren's syndrome.

摘要

CREST综合征是自身免疫性疾病硬皮病组中的一部分,该组疾病会导致身体不同部位的结缔组织增厚、硬化和紧绷,可能会引发复杂的病症。CREST综合征的特征是钙质沉着、雷诺现象、食管运动功能减退、指(趾)硬皮病和毛细血管扩张同时存在。一名72岁的白种女性因口腔疼痛、吞咽和进食困难、假牙不稳定及佩戴困难而被转诊至蒙扎的圣杰拉尔多医院。她之前被诊断出患有雷诺现象,之后又被诊断为CREST综合征。口外检查发现皮肤紧绷、增厚且僵硬,面部布满苍白色不规则斑、毛细血管扩张和肢端青紫。口内检查显示黏膜无改变,但可观察到舌僵硬,硬腭和前庭有一些红白相间的斑点。我们为该患者进行了干燥综合征的牙科治疗。干燥综合征的治疗是对症和经验性的,包括使用唾液分泌刺激剂、唾液替代品和辅助药物。基于牙膏和漱口水中局部氟化物的使用,并辅以氟凝胶和氟漆,牙科治疗和预防对于预防口干症的后果(如猖獗性龋齿)很重要。指导和加强口腔卫生,以及牙医的频繁牙科评估和管理,是维护患有进展为干燥综合征的CREST综合征且并发干燥综合征患者口腔健康的基本措施。

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