| Progressive overgrowth of the cerebriform connective tissue nevus in patients with Proteus syndrome. | |
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MedLine Citation:
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PMID: 20709429 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Proteus syndrome is a rare overgrowth disorder that almost always affects the skin. OBJECTIVE: Our purpose was to evaluate progression of skin lesions in patients with Proteus syndrome. METHODS: Skin findings were documented in 36 patients with Proteus syndrome. Progression of skin lesions in 16 of these patients was assessed by comparing photographs obtained on repeated visits for an average total duration of 53 months. RESULTS: The skin lesion most characteristic of Proteus syndrome, the cerebriform connective tissue nevus, showed progression in 13 children but not in 3 adults. The cerebriform connective tissue nevus progressed by expansion into previously uninvolved skin, increased thickness, and development of new lesions. Lipomas increased in size, number, or both in 8 of 10 children with lipomas. In contrast, epidermal nevi and vascular malformations generally did not spread or increase in number. LIMITATIONS: Only 3 adults with Proteus syndrome were evaluated longitudinally. CONCLUSION: The cerebriform connective tissue nevus in Proteus syndrome grows throughout childhood but tends to remain stable in adulthood. |
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Authors:
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Thomas M Beachkofsky; Julie C Sapp; Leslie G Biesecker; Thomas N Darling |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't Date: 2010-08-14 |
Journal Detail:
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Title: Journal of the American Academy of Dermatology Volume: 63 ISSN: 1097-6787 ISO Abbreviation: J. Am. Acad. Dermatol. Publication Date: 2010 Nov |
Date Detail:
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Created Date: 2010-10-18 Completed Date: 2010-11-16 Revised Date: 2011-12-21 |
Medline Journal Info:
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Nlm Unique ID: 7907132 Medline TA: J Am Acad Dermatol Country: United States |
Other Details:
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Languages: eng Pagination: 799-804 Citation Subset: IM |
Copyright Information:
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Copyright © 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved. |
Affiliation:
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Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Age Factors Child Child, Preschool Connective Tissue / growth & development, pathology* Disease Progression Epidermis / growth & development, pathology* Female Humans Infant Male Middle Aged Nevus / pathology* Proteus Syndrome / pathology* Skin Neoplasms / pathology* Young Adult |
| Grant Support | |
ID/Acronym/Agency:
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ZIA HG200328-07/HG/NHGRI NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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