| Rapidly progressing neurocutaneous melanosis (NCM) in a 49-year-old woman |
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Hee Jun Kim, Mi-Jung Kim, Yu Jung Kim, Jung Ho Han, Chang-Hun Huh, Hye Seung Lee, Jong Seok Lee |
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| 신경학적 증상의 급속한 악화가 동반된 성인에서의 신경피부흑색증 1예 |
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김희준, 김미정, 김유정, 한정호, 허창훈, 이혜승, 이종석 |
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| Abstract |
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Symptomatic neurocutaneous melanosis (NCM) rarely presents in adulthood. NCM is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi (CMN) combined with benign or malignant melanocytic proliferation in the leptomeninges. This syndrome is believed to be caused by an error in the morphogenesis of embryonal neuroectoderm. The prognosis of symptomatic NCM is extremely poor, even in the absence of malignancy. We describe a 49-year-old patient with a large CMN and numerous satellite nevi who presented with obstructive hydrocephalus followed by a rapid neurological deterioration over 4 months. The rarity of NCM in an adult hindered early diagnosis, and the diagnosis of NCM was made after an extensive work-up for other causes. In patients with acute neurologic symptoms and underlying CMN, the likelihood of NCM with or without leptomeningeal melanoma should be considered. |
| Key Words:
Neurocutaneous melanosis; Congenital melanocytic nevus |
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