Uncommon Oral Manifestations of Neurofibromatosis Type I – A Case Report
Abstract
Neurofibromatosis type I is an autosomal dominant genetic disorder. It is related to a mutation in the long arm of chromosome 17; however, it shows variable penetrance and about half of the cases have no family history of the disease. These patients present skin lesions such as cafe-au-lait spots, multiple neurofibromas, bone malformations, and central nervous system tumors as well. Diagnosis of NF-I & NF-II is based on clinical criteria. It is progressive in nature and one of its unique characters is the diversity of clinical expression from one patient to another and even within a family. NF-I also presents with certain oral manifestations which confers to the dentists a major responsibility for accurate diagnosis and report of the disease. We, herein, report a case of 35 year old female patient who was diagnosed incidentally for NF–I with unusual oral manifestations such as large tongue mass and hypo-plastic ramus & body of the right side of mandible.
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References
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