All of the neurocutaneous signs are seen in Neurofibromatosis Type 2, EXCEPT:
High-Yield Explanation
Shagreen patch is highly characteristic of tuberous sclerosis and is seen in 80% of patients. It occurs in early childhood and may be the first sign of disease. They are soft, flesh colored to yellow plaques with an irregular surface. It is most commonly seen in the lumbosacral region. Features of Neurofibromatosis 1 are: cafe au lait spots, neurofibromas or plexiform neuroma, freckling, optic glioma and Lisch nodules. Features of Neurofibromatosis 2 are: bilateral vestibular schwannomas, multiple meningioma, spinal ependymoma, astrocytoma, posterior subcapsular lens opacities and retinal hamaomas.