To the Editor: Dr Jean Latrelle, an eminent Parisian neurologist at the time of a meeting in Harriman, N.Y., started his discussion by saying, “Everything has been written, but not everything has been read”. I found the description of a patient with neurocutaneous melanosis by Oliveira de Andrade et al. (Epileptic Disord, 2004; 6: 145-52) interesting. For the past 30 years we have been followin...

A patient with multiple basal cell carcinoma syndrome, a symptom complex characteristized by nevoid basal cell carcinomas of the skin, jaw cysts, skeletal abnormalities, and hyporesponsiveness to parathormone is presented. In addition, the patient had a retroperitoneal lymphagiomyoma, a hamartomatous lesion, causing ureteral obstruction. The association of neuroectodermic syndromes and retroper...

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 presented with symptomatic hydrocephalus. A right frontal ventriculostomy was performed in the patie...

PURPOSE The presence of two or more Lisch nodules (melanocytic hamartomas of the iris) is one of seven diagnostic criteria for neurofibromatosis type 1 (NF1), a common monogenic disorder of dysregulated neurocutaneous growth. The hypothesis that Lisch nodules arise secondary to exposure to ultraviolet (UV) radiation from sunlight was investigated. METHODS Lisch nodule burden was mapped and qu...

The gross and microscopic features of an unusual case of neurocutaneous melanosis are described. This is an example of the rare association between a primary central nervous system malignant melanoma and a giant congenital melanocytic naevus of 'bathing trunk' distribution.

Neurocutaneous melanosis (NCM) is a rare neuroectodermal dysplasia characterized by large or multiple cutaneous congenital pigmented nevi and benign or malignant melanocytic tumors of the leptomeninges. Although the MR manifestations of this disease have been reported in a small series of cases, the usefulness of fluid-attenuated inversion recovery (FLAIR) MR findings has not been documented. W...

Many cutaneous conditions have associated ophthalmologic findings, which are important to recognize for both dermatologists and ophthalmologists. This review highlights some important ophthalmologic manifestations associated with neurocutaneous syndromes and inherited connective tissue diseases.