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[Neurocutaneous melanosis]
I W Husstedt1, K H Grotemeyer, M Galanski
1Klinik und Poliklinik für Neurologie der Westf. Wilhelmsuniversität Münster.
Abstract:
The authors report on a patient of 33 years of age suffering from neurocutaneous melanosis in whom two years after excision of a naevus pigmentosus et pilosus giganteus cellular proliferation of the melanoblasts in the area of the soft meninges was seen. This process led to the death of the patient within a few weeks' time despite intensive therapeutic measures, the manifestation being that of a generalised space-occupying growth in the CNS. Neuroradiological techniques can objectify the clinical pattern of signs of this very rare disease and may possibly enable an early diagnosis.
Insights
Neurocutaneous melanosis can lead to aggressive central nervous system (CNS) growth. Early diagnosis via neuroradiology is crucial for rare cases of meningeal melanoblast proliferation.
Area of Science:
- Neurology
- Dermatology
- Oncology
Background:
- Neurocutaneous melanosis is a rare congenital disorder.
- Giant congenital melanocytic nevi can have extracutaneous manifestations.
- Meningeal involvement in neurocutaneous melanosis is exceptionally rare and aggressive.