Extraoral: The darker pigmented, café au lait macules appear in childhood and increase in adulthood. Freckles may also be present in axillary and groin areas. Bone abnormalities. Seizures. Poor eye-hand coordination.
. Hypertension. Image of Lisch nodules depicts the freckling of the iris. Neurofibromatosis is diagnosed by the following:
(1) a first-degree relative with the condition, (2) six or more of the café au lait macules/freckling, (3) Lisch nodules, (4) optic glioma, (5) presence of two or more neurofibromas or one plexiform neurofibroma, and (6) bone changes. Life expectancy is decreased by about 15 years.
Intraoral: Multiple intra-oral neuromas: well-defined, painless, submucosal nodules. Tongue lesions appear as macroglossia. Intra-oral lesions can be subject to chronic trauma. Lesions may need to be removed if they interfere with oral function.