Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of congenital neurocutaneous melanosis

선천성 신경피부멜라닌증 1례

  • Ha, Sang Kyun (Department of Pediatrics, Sung-Ae General Hospital) ;
  • Lee, Jae Myoung (Department of Pediatrics, Sung-Ae General Hospital) ;
  • Kim, Eun Ryoung (Department of Pediatrics, Sung-Ae General Hospital) ;
  • Hwang, Ho (Department of Radiology, Sung-Ae General Hospital) ;
  • Lee, Hong Tak (Department of Dermatology, Sung-Ae General Hospital)
  • 하상균 (성애병원 소아과) ;
  • 이재명 (성애병원 소아과) ;
  • 김은령 (성애병원 소아과) ;
  • 황호 (성애병원 방사선과) ;
  • 이홍탁 (성애병원 피부과)
  • Received : 2005.09.05
  • Accepted : 2005.10.24
  • Published : 2006.02.15
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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.

신경피부멜라닌증은 피부와 중추신경계를 침범하는 드문 선천성 증후군으로 연수막에 멜라닌증 또는 흑색종과 동반하여 피부에 거대 또는 다수의 색소성 모반이 존재하며, 피부에서 악성 변화의 증거가 없고, 뇌수막 외에 다른 부위에 악성 흑색종의 증거가 없는 경우로 정의된다. 발생기전은 배아신경 외배엽의 형태발생 착오로 생기는 것으로 알려져 있다. 저자들은 복부, 흉부와 팔 전체에 검은색 모반이 덮여있고, 전신에 다양한 크기의 위성병변이 출생시부터 존재한 신생아를 경험하였다. 뇌 자기공명영상은 오른쪽 측두엽의 편도에 결절모양의 고신호 강도를 보였고, 지방억제 T1 강조영상은 연수막 주위의 고신호 강도를 보였다. 저자들은 거대 선천성 색소성 모반을 가진 신생아에서 뇌자기공명영상으로 진단받고 3차례의 소파술과 인공피부이식술을 받은 드문 선천성 증후군인 신경피부멜라닌증 환자 1례를 경험하고 문헌 고찰과 함께 보고하는 바이다.

Keywords

References

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