Covarrubias-Espinoza R, López-Armenta G, Hernández-Alvarado H, Rubio-López J, Hinojosa-Guadarrama F, Beltrán-Chanes JO, Pineda-Feliz H, Nuño-Vega MI, García-Hernández RJ, Cordero-Iribe ME

Language: Spanish
References: 12
Page: 85-88
PDF size: 487.71 Kb.

ABSTRACT

The neurocutaneousmelanosis (MNC) is an infrequent disease characterized by an excessive proliferation of melanocitic cells. characteristic cutaneous injuries are present at the time of the birth; nevertheless, the neurological manifestations of the disease show an ample variability with respect to the form of expression and in the time of appearance. There are neurological manifestation in minors of 2 years of age in 58% of the cases, and only 8% in patients greater than 20 years of age, a disease has been considered dominant that appears in any sex. Without showing racial predominance. The incidence of giant melanocitic nevus is 1: 20,000 to 50,000 born alive , and only one quarter presents/ displays central nervous system involvement. The located in the head, neck and posterior medial line can be associate with craneal and underlying medularleptomeningealmelanosis, which can not generate symptoms or give comunicant or noncomunicant hydrocephalia, focal convulsions, neurological defects, mental retardation and even melanomas. The finding of a giant congenital melanocytic nevus with clinical manifestations of the central nervous system, indicates the investigation of a neurocutaneous syndrome that involves the pigmentariy system. We present the clinical case of a boy diagnosed with neurocutaneous melanosis in evolution.

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