Document Detail


Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.
MedLine Citation:
PMID:  12140461     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Iftikhar Ahmed; Whitney D Tope; Terri L Young; Danielle M Miller; Kenneth E Bloom
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  47     ISSN:  0190-9622     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  2002 Aug 
Date Detail:
Created Date:  2002-07-25     Completed Date:  2002-08-14     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  S196-200     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, USA.
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MeSH Terms
Descriptor/Qualifier:
Eye Diseases / complications,  pathology
Female
Humans
Infant, Newborn
Lipomatosis / complications*,  pathology
Melanosis / complications*,  pathology
Neurocutaneous Syndromes / complications*,  pathology
Skin / pathology
Skin Diseases / complications*,  pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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