Abstract

ESTRELLA, V; QUIROGA, R; BERGERO, A  and  FERNANDEZ BUSSY, R. Neurofibromatosis: Plexiform Neurofibroma Localized  In  Mamma. A Propos Of A Case. Rev. argent. dermatol. [online]. 2010, vol.91, n.1, pp.00-00. ISSN 1851-300X.

The Neurofibromatosis was described by von Recklinghausen in 1882. It is a dominant autosomic genodermatosis with a high penetration, variable clinic and progressive course; it is calculate, that in 50% of the cases the origin is a spontaneous mutations (novo). The incidence is 1/3000 newly born. The clinical manifestations are: the compromise of tissues derived from neuroectoderm and mesoderm. It also has ocular symptoms, visceral injuries, neurological alterations and endocrinological pathologies. Soft pars, cutaneous tissue and bones are affected. At the present time two main clinical forms are admitted: the Classic Neurofibromatosis or type I, that represents 85-90% of the cases and the Neurofibromatosis type II (Neurofibromatosis of the bilateral hearing aid) that it ´s not so frequent.

Keywords : Neurofibromatosis Type I; Plexiform Neurofibroma; Von Recklinghausen.

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