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Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.

Publication ,  Journal Article
Ahmed, I; Tope, WD; Young, TL; Miller, DM; Bloom, KE
Published in: J Am Acad Dermatol
August 2002

We describe a white female infant with neurocutaneous melanosis (NCM) and encephalocraniocutaneous lipomatosis (ECCL). Multiple, giant and small congenital melanocytic nevi (CMN) were observed on the head, neck and trunk and involved 70% of body surface area. Histologic examination of several CMN revealed atypical nodular proliferations of dermal nevomelanocytes. In a small (<1 cm) truncal CMN, single and dyscohesive intraepidermal nests of atypical nevomelanocytes simulating a superficial spreading melanoma, were observed. The placenta was grossly normal and histologically demonstrated multiple banal appearing nevomelanocytes within the stroma of its villi. At the 17-month follow-up no evidence of primary or metastatic melanoma was present. This previously undescribed association of NCM, ECCL and placental nevomelanocytes provides strong support for the hypothesized causal role of anomalous neural crest morphogenesis and migration in the development of all three disorders. The genetic mechanism underlying these complex birth defects has been hypothesized to result from the action of lethal autosomal dominant genes surviving by mosaicism.

Duke Scholars

Published In

J Am Acad Dermatol

DOI

ISSN

0190-9622

Publication Date

August 2002

Volume

47

Issue

2 Suppl

Start / End Page

S196 / S200

Location

United States

Related Subject Headings

  • Skin Diseases
  • Skin
  • Neurocutaneous Syndromes
  • Melanosis
  • Lipomatosis
  • Infant, Newborn
  • Humans
  • Female
  • Eye Diseases
  • Dermatology & Venereal Diseases
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Ahmed, I., Tope, W. D., Young, T. L., Miller, D. M., & Bloom, K. E. (2002). Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis. J Am Acad Dermatol, 47(2 Suppl), S196–S200. https://doi.org/10.1067/mjd.2002.110073
Ahmed, Iftikhar, Whitney D. Tope, Terri L. Young, Danielle M. Miller, and Kenneth E. Bloom. “Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.J Am Acad Dermatol 47, no. 2 Suppl (August 2002): S196–200. https://doi.org/10.1067/mjd.2002.110073.
Ahmed I, Tope WD, Young TL, Miller DM, Bloom KE. Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis. J Am Acad Dermatol. 2002 Aug;47(2 Suppl):S196–200.
Ahmed, Iftikhar, et al. “Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.J Am Acad Dermatol, vol. 47, no. 2 Suppl, Aug. 2002, pp. S196–200. Pubmed, doi:10.1067/mjd.2002.110073.
Ahmed I, Tope WD, Young TL, Miller DM, Bloom KE. Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis. J Am Acad Dermatol. 2002 Aug;47(2 Suppl):S196–S200.
Journal cover image

Published In

J Am Acad Dermatol

DOI

ISSN

0190-9622

Publication Date

August 2002

Volume

47

Issue

2 Suppl

Start / End Page

S196 / S200

Location

United States

Related Subject Headings

  • Skin Diseases
  • Skin
  • Neurocutaneous Syndromes
  • Melanosis
  • Lipomatosis
  • Infant, Newborn
  • Humans
  • Female
  • Eye Diseases
  • Dermatology & Venereal Diseases