Facial plexiform neurofibroma in a 13-year-old girl with neurofibromatosis-1

Neurofbromatosis (NF) is an autosomal dominant disorder that affects the bone, the nervous system, soft tissue and the skin. NF is a neurocutaneous condition that can involve almost any organ system existing in two subtypes. NF1 is the most common subtype and is referred to as peripheral NF. Plexifo...

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Bibliographic Details
Published in:Journal of Indian Academy of Oral Medicine and Radiology 2012-07, Vol.24 (3), p.243-249
Main Authors: Misra, Satya, Maragathavalli, G, Baskaran, Pavitra, Rastogi, Varun
Format: Article
Language:English
Subjects:
Café au lait macules
Plexifonn neurofibroma
Teenage girls
Type I neurofibromatosis
von Recklinghausen′s disease
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Summary:Neurofbromatosis (NF) is an autosomal dominant disorder that affects the bone, the nervous system, soft tissue and the skin. NF is a neurocutaneous condition that can involve almost any organ system existing in two subtypes. NF1 is the most common subtype and is referred to as peripheral NF. Plexiform neurofibromas are diffuse, elongated fibromas coursing along the nerves. These lesions frequently involve the trigeminal or upper cervical nerves- Though plexiform neurofibroma occurs in only 5% of patients with NF 1, it is considered pathognomonic feature of NF1. We present a case of plexiform neurofibroma in the left side of the face in a 13-year-old girl with NF1.
ISSN:0972-1363
0975-1572
DOI:10.4103/0972-1363.166294