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Chapter 62. Neurofibromatosis

Definition

A constellation of lesions that represent disorders of melanocytes (e.g., axillary “freckles” and cafĂ© au lait “spots”) and of proliferations of Schwann cells (e.g., papules, nodules, and tumors of neurofibroma). The cutaneous lesions may be accompanied by neoplasms with neural differentiation that are situated in nerves (e.g., acoustic neuroma) and in the brain (e.g., gliomas), as well as by manifestations of various kinds in the eyes (e.g., Lisch nodules) and bones (e.g., scoliosis).

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