Resumo

VARGAS FLORES, Tania  e  ROJAS MAMANI, Christhian Matteuss. NEUROFIBROMATOSIS TIPO I (NF-1). Rev. Act. Clin. Med [online]. 2014, vol.45, pp. 2400-2405. ISSN 2304-3768.

ABSTRACT Neurofibromatosis is an inherited disease autosomal dominant character altering development of cells belonging to the neural crest, which can manifest itself in different ways. Neurofibromatosis type I is also called Von Recklinghausen's disease or peripheral neurofibromatosis occurs in 1 in 3,000 births and is characterized by the association of neurological and dermatological disorders. There are criteria that help to diagnose this disease, seen as major signs latte stains that appear on the skin, the sign of Crowe, ocular and dermal neurofibromas level Lisch nodules. In turn minor signs mentioned are bone deformities, oral manifestations of soft tissue level and the difficulty of learning. For the clinical features that this disease presents is necessary to make a differential diagnosis of certain syndromes, such as Leopard syndrome, Proteus syndrome and McCune Albright syndrome. Among the complications of the disease, it is mentioned cancer as a result of malignant transformation of a neurofibroma

Palavras-chave : Neurofibromatosis peripheral; Von Recklinghausen's disease; Neurocutaneous syndrome.