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遺傳性牙齦纖維瘤-病例報告

Hereditary Gingival Fibromatosis-Report of Two Cases

摘要


遺傳性牙齦纖維瘤為一種較為罕見的牙齦增生疾病,發生機率約為七十五萬分之一,男女比例並無差異。牙齦增生的病徵在臨床上不算少見,常常是由發炎反應所衍生,其它較可能引起牙齦增生的因子尚有因使用藥物引起,或與遺傳有關,另外是否有腫瘤(如單核球血癌)的因素也必須考量。本文報告一對姐妹,在不同時間皆因為口內牙齦組織增生問題前來求診,其家族史裡的母親、阿姨與外祖母都有牙組織增生的現象。經評估後,兩姐妹都沒有服用可能引發牙齦增生的藥物,影像學及組織細胞學檢查也沒有腫瘤的發現。生理檢查發現妹妹的身體四肢的毛髮量較多之外,其他兩人的關節活動與聽力、智力觀察皆屬正常範圍。遺傳性牙齦纖維瘤常因為牙齦向牙冠方向的過度增生,進而造成口腔清潔、咀嚼的困難以及美觀上的不雅,造成社交上的障礙,並引發心理疾病。遺傳性牙齦纖維瘤的治療方式目前大多以手術切除為主,並輔以口腔衛生指導,降低復發機率。本文將就這罕見的病例,提出鑑別診斷以及治療方式,並加以討論。

並列摘要


Hereditary Gingival Fibromatosis is a rare gingival hyperplasia disease which prevalence is about 1 : 750000 and without gender predominant. Gingival hyperplasia disease usually comes from gingival inflammation; Other factors like specific medicine side effect (eg: Phenytoin, Cyclosporine, Nifedipine ) or genetics influence(eg: Zimmennan-Laband syndrome, Jones symdrome, HGF-hypertrichosis syndrome, Ramon syndrome, Rutherford's syndrome) or malignant tumor induced (eg: Monocytic leukemia) may cause gingival hyperplasia. This report includes two sisters who came to our clinic for gingival hyperplasia problem at different appointment. A family history of gingival hyperplasia (their mother, aunt and maternal grandmother) was noted. There was no specific medicine history nor image or histopathologic malignant tumor finding on them Physical examination found no abnormality in joint activity nor intelligence except slight increase amount in extremities. Patients with Hereditary Gingival Fibromatosis complain ill-looking gingival appearance and periodontal problems occur due to difficult to maintain oral hygiene. Biting pain happens when their teeth contact onto hyperplasia gingiva. Surgical excision of the hyperplasia tissue to restore the gingival contours is the main treatment option of hereditary gingival fibromatosis. Oral hygiene instruction with discoloring agent may reduce recurrence. We present these rare cases for differential diagnosis、treatment and discussion.

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