Oral manifestations of Type I Neurofibromatosis in a family
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Khan, Mubeen; Ohri, Neera
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Aquest document és un/a article, creat/da en: 2011
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Neurofibroma is a benign peripheral nerve sheath tumor. It is one of the most frequent tumors of neural origin and
its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type I (NF-I). Neurofibromatosis
type I is an autosomal dominantly inherited disease due to an alteration in the long arm of chromosome 17. About
50% of NF-I patients have no family history of the disease. NF-I patients have skin lesions (café au lait spots and
neurofibromas) as well as bone malformations and central nervous system tumors. Diagnosis is based on a series of
clinical criteria. NF-I presents with certain definite oral manifestations which confers to the dentists a major responsibility for accurate diagnosis and report of the disease. The paper aims to highlight the role of an oral diagnostician
in first recognition of NF-I in a family via manifestations in the oral cavity |
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