Aggressive Periodontitis in a Patient with Ehlers-Danlos Syndrome: Report of a Case

Ehlers-Danlos Syndrome的兒童患者合併快速擴散型牙周病－病例報告

施如軒(Ju-Hsuan Shih) ； 汪靜蓉(Ching-Jung Wang)

《臺灣兒童牙醫學雜誌》 6卷4期 (2006/12) Pp. 353-361

https://doi.org/10.6319/TJPD.2006.6(4).5

摘要

Ehlers-Danlos症候群(EDS)是一種影響結締組織的遺傳性疾病，臨床的特徵是皮膚有高度的延展性、關節有高度的活動性、且組織容易擦傷結疤。EDS的發生率1/5,000到1/10.000，診斷通常是靠臨床特徵而定。一位12歲7個月的中國女孩來台北長庚醫院兒童牙科，希望得到快速擴散型牙周病的相關諮詢與治療。經過口腔檢查發現她有許多蛀牙、一些缺牙及全口牙齦發炎腫大現象。X光檢查發現在前牙及大臼齒區有多處的骨質缺損及多生牙，而臨床檢查則發現她有典型的EDS症狀，包括：明顯的傷疤、高度活動性的關節，高度延展性的皮膚。我們提供的治療包括：口腔衛教、全口第一階段的牙周病治療、移除無法治療的牙齒、填補蛀牙及給予抗生素。經過五個月的牙科治療，患者的口腔衛生進步許多、沒有新的蛀牙、且拔牙的傷口癒合良好，至於急性牙齦發炎的情況，除了上顎正中門牙區外，其餘皆已緩解。本病例報告是關於一位女童因快速擴散型牙周病的發現，進而診斷出其潛在的系統性疾病(EDS)，並探討相關的牙科處置方式。

關鍵字

Ehlers-Danlos症候群 ； 快速擴散型牙周病 ； 提早掉落的恆牙

並列摘要

Ehlers-Danlos Syndrome (EDS) is a heterogeneous group of heritable connective tissue disorders that is characterized by skin extensibility, joint hypermobility, and tissue fragility. The prevalence of EDS has been estimated to be between 1 in 5,000 and 1 in 10,000. A 12-year 7-month-old Chinese girl was brought to pediatric department of Taipei Chang-Gung Memorial Hospital for the evaluation and treatment of aggressive periodontitis. Oral examinations revealed multiple dental caries, missing teeth, and generalized gingival swelling. Multiple bony defects over incisor and molar regions and supernumerary teeth were demonstrated in the radiographic examination. Clinical examination showed the typical cutaneous features of EDS with extensive scarring, hypermobility of joints, and hyper-extensibility of skin. Treatment modalities included oral hygiene instruction, phase-one periodontal therapy, extraction of the hopeless teeth, restorations of the carious teeth, and systemic antibiotics therapy. After 5-month dental treatment, the patient's oral hygiene was improved significantly, no carious lesions were noted, the healing of extracted wound was good, and signs of acute inflammation were subsided expect the region over maxillary central incisors. This case report presents an example of how periodontal involvement may lead to the diagnosis of an underlying systemic condition.

並列關鍵字

Ehlers-Danlos Syndrome ； Aggressive Periodontitis ； Premature Loss of Permanent Tooth