neurocutaneous syndromes/melasma

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Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma.

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OBJECTIVE To describe the features of phacomatosis pigmentovascularis (cesioflammea type). METHODS Noninterventional retrospective case series composed of 7 patients. RESULTS Nevus flammeus combined with ipsilateral ocular melanocytosis or melanosis was seen in all 7 patients. Additional

Intramedullary and retroperitoneal melanocytic tumor associated with congenital blue nevus and nevus flammeus: an uncommon combination of neurocutaneous melanosis and phacomatosis pigmentovascularis--case report.

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Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived

Dandy-Walker malformation and neurocutaneous melanosis in a three-month-old infant.

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Dandy-Walker Malformation (DWM) is a rare congenital malformation of the brain. It is characterized by cystic enlargement of the fourth ventricle which is communicating with an enlarged posterior fossa, cerebellar dysgenesis, high tentorial insertion and hydrocephalus. Neurocutaneous Melanosis (NCM)

Neurocutaneous melanosis in a newborn with giant congenital melanocytic nevus.

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Neurocutaneous melanosis (NCM) is known as a rare phakomatose characterised by large or numerous pigmented congenital nevi associated with leptomeninges melanin-containing deposits. We report a case of a newborn presenting at birth with a giant nevus covering about 40% of the total body surface. MRI

Neurocutaneous melanosis: surgical pathological features of an apparently hamartomatous lesion in the amygdala.

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Neurocutaneous melanosis (NCM) is a rare, congenital phakomatosis characterized by the presence of congenital melanocytic nevi and a benign or malignant pigmented cell tumor of the leptomeninges of the CNS. Here the authors report the surgical pathological features of a lesion in the left amygdala

Neurocutaneous melanosis.

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Neurocutaneous melanosis (NCM) is a rare phakomatosis characterized by a focal or diffuse proliferation of melanin-producing cells in both the skin and the leptomeninges. This syndrome is believed to result from an error in the morphogenesis of embryonal neuroectoderm. Two-thirds of patients with

Characteristic MR Imaging Findings of Neurocutaneous Melanosis. A Case Report.

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Neurocutaneous melanosis is a type of phakomatosis characterised by dermal, leptomeningeal and parenchymal melanocytic naevi. Here we describe a case of a 13-year-old boy with dermal and brain parenchymal melanosis without any leptomeningeal melanosis.

[Neurocutaneous melanosis--a case report].

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Neurocutaneous melanosis is a rare congenital phacomatosis characterized by the presence of large or multiple congenital melanocytic nevi and benign or malignant pigmented cell tumors of the leptomeninges. A 14-month-old boy was admitted with a recent history of vomiting and drowsiness. He was found

Neurocutaneous melanosis presenting as chronic partial epilepsy.

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BACKGROUND Neurocutaneous melanosis (NCM) is a rare neurocutaneous syndrome characterized by the presence of multiple congenital melanocytic nevi (CMN) and the proliferation of melanocytes in the central nervous system, usually involving the leptomeninges. Chronic partial epilepsy as a sole

Neurocutaneous melanosis.

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Neurocutaneous melanosis is a rare neurocutaneous syndrome that is associated with a high rate of mortality early in life. Individuals with large cutaneous melanocytic nevi (LCMN) are at risk, especially when the nevi are posterior, midline and accompanied by satellite nevi. Disrupted production and

Neurocutaneous melanosis presenting with hydrocephalus. Case report and review of the literature.

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Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant melanocytic tumors of the leptomeninges. The authors report the case of a 5-month-old girl with congenital giant melanocytic nevi who

Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report.

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The authors report the investigation, treatment, and progress of a unique case of neurocutaneous melanosis with syringomyelia. The suspected etiology, the pathology, and the variable clinical manifestations of this rare phakomatosis are recounted. The potential for malignant change and the possible

[Neurocutaneous melanosis].

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Neurocutaneous melanosis is a sporadic neurocutaneous syndrome characterized by the presence of giant or multiple melanocytic nevi and excessive proliferation of melanotic cells in the central nervous system. Patients usually consult dermatologists because the congenital cutaneous nevi. Most

Ataxia telangiectasia, Menkes kinky hair disease and neurocutaneous melanosis.

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This article explores three neurocutaneous syndromes (NCSs), i.e. genetic disorders producing developmental abnormalities of the skin and an increased risk of neurological complications. In this review, different aspects of ataxia telangiectasia, Menkes kinky hair disease and neurocutaneous

Fundus features of a case of neurocutaneous melanosis.

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A 5-year-old girl with giant cutaneous congenital melanocytic nevi was examined because of right esotropia. She had multiple uveal coloboma-like lesions of various sizes and irregular areas of retinal pigment epithelial alterations in the right fundus. Magnetic resonance imaging studies demonstrated

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