Rapidly progressing neurocutaneous melanosis NCM in a 49-year-old woman / 대한내과학회지

ABSTRACT

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.