Neurofibromatosis type I with periodontal manifestation. A case report and literature review.
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.
Cunha
K S G
KS
Graduate Student, Fluminense Federal University (UFF), School of Medicine, Department of Pathology, Rio de Janeiro, Brazil. karingoncalves@terra.com.br
Barboza
E P
EP
Dias
E P
EP
Oliveira
F M
FM
D001706:Biopsy; D005260:Female; D005887:Gingival Neoplasms / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007180:Incisor / Q000473:pathology; D008334:Mandible; D008875:Middle Aged; D009455:Neurofibroma / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000473:pathology*