Multiple randomly distributed, flesh-colored nodules were noted on the trunk, arms, and face of a 62-year-old man. The lesions measured 0.5 to 1.0 cm and appeared slightly pedunculated. He had had the lesions since he was a teenager; they were not painful. Tissue biopsy confirmed the suspected diagnosis of neurofibromatosis type I.
Neurofibromatosis may occur sporadically or follow a familial distribution. Type I manifests as flesh-colored nodules (neurofibromas) randomly distributed throughout the body. Type II manifests as axillary freckling and skin pigmentation disruptions (caf au lait spots). It is associated with eighth cranial nerve tumors of multiple endocrine neoplasia.
Systemic complications that have been associated with neurofibromatosis include meningiomas, gliomas, mental retardation, obstructive hydrocephalus, and peripheral sarcomas.