Cunha K S G, Barboza E P, Dias E P, Oliveira F M
Graduate Student, Fluminense Federal University (UFF), School of Medicine, Department of Pathology, Rio de Janeiro, Brazil.
Br Dent J. 2004 Apr 24;196(8):457-60. doi: 10.1038/sj.bdj.4811175.
The term neurofibromatosis (NF) is used for a group of genetic disorders that primarily affect the cell growth of neural tissues. Neurofibromatosis type 1 (NF1), also known as von Recklinghausen's disease, is the most common type of NF and accounts for about 90% of all cases. It is one of the most frequent human genetic diseases, with a prevalence of one case in 3,000 births. The expressivity of NF1 is extremely variable, with manifestations ranging from mild lesions to several complications and functional impairment. Oral manifestations can be found in almost 72% of NF1 patients. A case of a NF1 patient with a gingival neurofibroma in the attached gingiva of the lingual aspect of the lower central incisors is presented. The lesion was nodular, with sessile base, non-ulcerated, non-painful, with normal colour and measured 1 cm in diameter. An excisional biopsy of the oral lesion was performed. Histopathological and immunohistochemical analysis confirmed the clinical hypothesis of neurofibroma. Because NF1 is one of the most common genetic diseases and oral manifestations are very common, dentists should be aware of the characteristics of this disease.
神经纤维瘤病(NF)一词用于指代一组主要影响神经组织细胞生长的遗传性疾病。1型神经纤维瘤病(NF1),也称为冯雷克林霍增氏病,是最常见的NF类型,约占所有病例的90%。它是最常见的人类遗传疾病之一,发病率为每3000例出生中有1例。NF1的表现度极具变异性,表现范围从轻度病变到多种并发症和功能障碍。几乎72%的NF1患者会出现口腔表现。本文介绍了1例NF1患者,其在下颌中切牙舌侧附着龈处有牙龈神经纤维瘤。病变呈结节状,有蒂状基部,无溃疡,无疼痛,颜色正常,直径1厘米。对口腔病变进行了切除活检。组织病理学和免疫组织化学分析证实了神经纤维瘤的临床诊断。由于NF1是最常见的遗传疾病之一且口腔表现非常常见,牙医应了解这种疾病的特征。