Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Neurocutaneous melanosis.

Hanspaul S Makkar1, Ilona J Frieden

  • 1Department of Dermatology and Pediatrics, University of California, San Francisco, CA, USA.

Seminars in Cutaneous Medicine and Surgery
|August 7, 2004
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Hemangiomas: Beyond the Skin.

Pediatrics in review·2026
Same author

A Case of Encephalocraniocutaneous Lipomatosis (ECCL) With Atypical Clinical Presentation Diagnosed on Molecular Testing: FGFR1 ECCL Tumor Risk.

American journal of medical genetics. Part A·2026
Same author

Alpelisib, a PI3Kα inhibitor, effectively treats vascular anomalies with diverse genotypes and phenotypes.

Blood vessels, thrombosis & hemostasis·2026
Same author

Incontinence-associated dermatitis: Not just babies.

Journal of the American Academy of Dermatology·2025
Same author

Paradigm shift in the management of infantile hemangiomas in the beta-blocker era: A retrospective cohort study.

Journal of the American Academy of Dermatology·2025
Same author

Updated Classification of Vascular Anomalies. A living document from the International Society for the Study of Vascular Anomalies Classification Group.

Journal of vascular anomalies·2025

Neurocutaneous melanosis (NCM) is a rare congenital disorder involving melanocytic nevi and leptomeningeal melanocyte proliferation. Early diagnosis and management are crucial due to the risk of CNS melanoma and poor prognosis in symptomatic patients.

Area of Science:

  • Genetics and Developmental Biology
  • Pediatric Neurology
  • Dermatology

Background:

  • Neurocutaneous melanosis (NCM) is a rare congenital disorder.
  • Characterized by congenital melanocytic nevi and leptomeningeal melanocyte proliferation.
  • Pathogenesis may involve errors in neural ectoderm morphogenesis and HGF/SF signaling.

Purpose of the Study:

  • To review the key features, pathogenesis, clinical manifestations, and prognosis of NCM.
  • To highlight the association with CNS anomalies and the risk of melanoma.
  • To discuss the diagnostic challenges and management strategies.

Main Methods:

  • Literature review of NCM cases and relevant research.
  • Analysis of clinical presentations, imaging findings, and outcomes.

Related Experiment Videos

  • Synthesis of information on embryogenesis and molecular pathways.

    • Main Results:

    • NCM presents with large or multiple nevi, often with posterior axial distribution.
    • Neurologic symptoms, typically within two years, relate to increased intracranial pressure.
    • CNS melanoma develops in approximately half of patients; prognosis is poor.
    • Associated CNS anomalies like Dandy-Walker complex are reported.

    Conclusions:

    • NCM is a complex disorder with significant neurological risks, including melanoma.
    • Early recognition and monitoring are vital for improving patient outcomes.
    • Further research is needed to clarify the significance of MRI findings in asymptomatic individuals.