Neurocutaneous melanosis in a woman with multiple brain melanocytomas, cutaneous melanocytosis and oral involvement

A 34-year-old female was referred to us for a consultation of her dermatological lesions (pigmented lesions present in her oral cavity and on her right shoulder) in May 2007. These lesions had been present since childhood. Recently, the patient had developed seizures and a headache. An MRI of the brain showed the presence of two intracranial masses. The intracranial tumours were surgically removed whereas skin and mucosal lesions were biopsied. Histological findings of brain tumours were consistent with a diagnosis of "melanocytoma" while cutaneous lesions presented "benign dermal melanocytic infiltrations". Whole brain irradiation was performed. After 3 months a new melanocytic skin lesion appeared on the scalp with histological picture similar to the other cutaneous ones. At the 5-year follow-up examination no recurrence of intracranial tumour or other skin or mucosal lesions were registered. According to the clinical and histological findings, we classify our case as a form of neurocutaneous melanosis in a young adult patient and we present it for the rarity of this syndrome, for the difficulty of the diagnosis, for the potential aggressive behaviour of intracranial lesions that necessitates a constant attentive follow-up and for the unusual feature of new developing skin lesion during the course of the disease.