Familiar multiple neurofibromatosis: report of case

RESUMO

The study presents a case of neurofibromatosis familiar involving a 4-year-old boy and his family. The lesions that characterize this syndrome are neurofibromas distributed over the entire body, associated to "café-au-lait" spots. This occurs due to genetic disorder and it is an autossomal dominant trait. The growth of the refered process was showed asymptomatically in the left side of the patient's face. They also had exophthalmia, expansion of the upper and lower maxilla in the same side and "café-au-lait" spots. His father also had these spots and multiple neurofibromas without sympton. According to the history, clinical and radiographic aspects it was diagnosed that familiar multiple neurofibromatosis (von Recklinghausen's disease) must be followed, as the neurofibroma might suffer malignant degeneration.