Large congenital melanotic nevi in an extremity with neurocutaneous melanocytosis Journal Article


Authors: Becher, O. J.; Souweidane, M.; Lavi, E.; Kramer, K.; Lis, E.; Marghoob, A. A.; Khakoo, Y.
Article Title: Large congenital melanotic nevi in an extremity with neurocutaneous melanocytosis
Abstract: A 14-day-old boy presented with a large congenital melanocytic nevus over his left thigh with approximately 17 satellite nevi distributed over the rest of his skin surface. Six weeks later, he developed generalized tonic-clonic seizures and additional satellite nevi became apparent (n > 20). A subsequent brain magnetic resonance imaging demonstrated right temporal T1 hyperintense signal abnormality. At 4 months of age the patient underwent a lumbar puncture that was normal without evidence of melanocytes or tumor. Nevertheless, a few days later he underwent resection of his right medial temporal lesion which demonstrated melanocytosis in the temporal lobe as well as melanocytosis in subependymal areas in other parts of the brain and ventricles, confirming the suspected diagnosis of neurocutaneous melanocytosis. Our case supports previous studies that conclude that the number of satellite nevi is a greater predictor of neurocutaneous melanocytosis than is the location of large congenital melanocytic nevus. In our case, cerebrospinal fluid studies were not reliable even in the face of florid neurocutaneous melanocytosis involving the leptomeninges and ventricles. © 2009 The Authors.
Keywords: case report; nuclear magnetic resonance imaging; magnetic resonance imaging; melanoma; skin neoplasms; melanocytes; subventricular zone; etiracetam; oxcarbazepine; phenobarbital; topiramate; add on therapy; brain; brain ventricle; cerebrospinal fluid; grand mal seizure; limb; lumbar puncture; melanocytic nevus; melanocytosis; phakomatosis; skin surface; temporal lobe; thigh; tonic clonic seizure; epilepsy, tonic-clonic; infant; infant, newborn; meningeal neoplasms; meninges; neurocutaneous syndromes; nevus, pigmented
Journal Title: Pediatric Dermatology
Volume: 26
Issue: 1
ISSN: 0736-8046
Publisher: Blackwell Scientific Publications  
Date Published: 2009-01-01
Start Page: 79
End Page: 82
Language: English
DOI: 10.1111/j.1525-1470.2008.00828.x
PUBMED: 19250413
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 3" - "Export Date: 30 November 2010" - "CODEN: PEDRD" - "Source: Scopus"
Altmetric Score
MSK Authors
  1. Kim Kramer
    168 Kramer
  2. Eric Lis
    102 Lis
  3. Yasmin Khakoo
    99 Khakoo
  4. Ashfaq A Marghoob
    385 Marghoob
  5. Oren Josh Becher
    25 Becher