Year : 2016

Volume : Volume 6

Issue : Issue 1

Address for correspondence :
Dr. Sairam V
drsai1@rediffmail.com

Abstract

Background:  The term neurofibromatoses is applied to a group of autosomal dominant disorders characterized by neuroectodermal tumors involving several parts of the body. Among the eight types recognized, Neurofibromatosis Type-1 is common without racial differences. It is chiefly characterized by six or more cafe´au-lait macules, two or more neurofibromas, freckling in the axillary or inguinal region and Lisch nodules over the iris. Oral neurofibromas are extremely variable and rare, posing a diagnostic challenge to oral healthcare providers. We present a rare case of Neurofibromatosis type- 1 with oral nuerofibromas (two) in the hard palate, presence of cafe au lait (coffee with milk) pigmentation, discrete multiple nodules over different regions of the body, two Lisch’s nodules of Iris. Clinical presentation of this patient is in accordance with the diagnostic criteria given by the National Institutes of Health Criteria for Neurofibromatosis Type 1.

Keywords: Cafe´-Au-Lait Macules, Lisch’s Nodules, Oral Neurofibroma, Neurofibromatosis Type-1.