PMID- 15295923
OWN - NLM
STAT- MEDLINE
DCOM- 20041116
LR  - 20051116
IS  - 1085-5629 (Print)
IS  - 1085-5629 (Linking)
VI  - 23
IP  - 2
DP  - 2004 Jun
TI  - Neurocutaneous melanosis.
PG  - 138-44
AB  - Neurocutaneous melanosis (NCM) is a rare congenital disorder characterized by the 
      presence of large or multiple congenital melanocytic nevi in association with 
      benign or malignant proliferation of melanocytes in the leptomeninges. NCM is 
      believed to occur as a consequence of an error in morphogenesis in neural 
      ectoderm in the developing embryo. Animal models suggest that aberrant expression 
      of the hepatocyte growth factor/scatter factor (HGF/SF) may be involved in the 
      pathogenesis in the NCM. While the majority of patients with NCM have large 
      congenital melanocytic nevi in a posterior axial distribution, a significant 
      proportion of patients present with multiple smaller nevi in the absence of a 
      single larger lesion. Neurologic manifestations generally occur within the first 
      two years of life, and are often related to increased intracranial pressure. 
      Associated structural anomalies of the CNS have been reported in NCM, 
      particularly the Dandy-Walker complex. The long-term clinical significance of 
      characteristic magnetic resonance findings in neurologically asymptomatic 
      patients is unclear. Approximately half of NCM patients develop CNS melanoma. The 
      prognosis of symptomatic patients remains poor.
FAU - Makkar, Hanspaul S
AU  - Makkar HS
AD  - Department of Dermatology and Pediatrics, University of California, San 
      Francisco, CA, USA.
FAU - Frieden, Ilona J
AU  - Frieden IJ
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Semin Cutan Med Surg
JT  - Seminars in cutaneous medicine and surgery
JID - 9617260
SB  - IM
MH  - Diagnosis, Differential
MH  - Humans
MH  - Hydrocephalus/*diagnosis/etiology
MH  - Magnetic Resonance Imaging
MH  - Melanosis/complications/*diagnosis
MH  - Neurocutaneous Syndromes/complications/*diagnosis
MH  - Prognosis
RF  - 39
EDAT- 2004/08/07 05:00
MHDA- 2004/11/17 09:00
CRDT- 2004/08/07 05:00
PHST- 2004/08/07 05:00 [pubmed]
PHST- 2004/11/17 09:00 [medline]
PHST- 2004/08/07 05:00 [entrez]
AID - S1085-5629(04)00008-2 [pii]
AID - 10.1016/j.sder.2004.01.007 [doi]
PST - ppublish
SO  - Semin Cutan Med Surg. 2004 Jun;23(2):138-44. doi: 10.1016/j.sder.2004.01.007.