All issues > Volume 49(2); 2006

Case Report

Korean J Pediatr. 2006;49(2):212-216. Published online February 15, 2006.

A case of congenital neurocutaneous melanosis

Sang Kyun SK Ha¹, Jae Myoung JM Lee¹, Eun Ryoung ER Kim¹, Ho H Hwang², Hong Tak HT Lee³

¹Department of Pediatrics, Sung-Ae General Hospital, Seoul, Korea
¹Department of Pediatrics Sung-Ae General Hospital, Seoul, Korea
¹Department of Pediatrics Sung-Ae General Hospital, Seoul, Korea
²Department of Radiology Sung-Ae General Hospital, Seoul, Korea
³Department of Dermatology, Sung-Ae General Hospital, Seoul, Korea

Correspondence Eun Ryoung ER Kim ,Email: eunicu@hanmail.net

Abstract

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign pigment cell tumors of the leptomeninges. Neurocutaneous melanosis is thought to represent an error in the morphogenesis of embryonal neuroectoderm. We experienced a neonate who presented with giant, dark colored pigmented nevi covering chest, abdomen, neck and arms, with satellite lesions. Magnetic resonance image showed a nodular hyperintense lesion in the amygdala of the right temporal lobe, and T1-weighted images showed hyperintensities in the adjacent leptomeninges. We report a rare case of neurocutaneous melanosis with a brief review of related literature.

Keywords :Neurocutaneous melanosis , Newborn