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A case of congenital neurocutaneous melanosis

Korean Journal of Pediatrics 2006;49(2):212-216.
Published online February 15, 2006.
A case of congenital neurocutaneous melanosis
Sang Kyun Ha1, Jae Myoung Lee1, Eun Ryoung Kim1, Ho Hwang2, Hong Tak 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
선천성 신경피부멜라닌증 1례
하상균1, 이재명1, 김은령1, 황호2, 이홍탁3
1성애병원 소아과
1성애병원 소아과
1성애병원 소아과
2성애병원 방사선과
3성애병원 피부과
Correspondence: 
Eun Ryoung 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.
Key Words: Neurocutaneous melanosis , Newborn


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