Neurocutaneous melanosis

Won, Jin Hyoung; Ahn, Sung Ku; Kim, Soo Chan; Lee, Seung Hun

Abstract

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital pigmented nevi and a benign or maigrant pigment cell tumor of the leptomeninges. The s ndrome is thought to represent an erroi in the morphogenesis of the embryonal neuroectoderm. Even in the absence of melanorra, symptomatic neurocut.aneous melanosis has extremely poor prognosis. We report a case of neiirocutaneous melanosis in 38-year-old male. He was noted at birth to have a giant pigmented nevus on the bathing trunk area. He presneted with severe headache. Brain CT sca n was performed and 4 x 4cm sized well demarcated contrast enhanced mass was seeii in the right frontal lobe. Histopathologically, the tumor was composed of malignant melanoma cells. Multiple skin biopsies werperformed on the giant pigmented nevus and small nevi. All of the specimens revealed patterns typical of cornpound nevi. Dermatologists following patients with large or multiple congenial pigmented nevi should be aware of this condition to aid in prompt diagnosis.