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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
5
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pubmed:dateCreated |
1990-1-23
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pubmed:abstractText |
The 5 major cutaneous defects of development found in focal dermal hypoplasia, an ectomesodermal dysplasia syndrome, are: aplasia cutis congenita, multiform atrophy-like areas, striate, papillomatous, and lipomatous lesions of skin. Subepidermal lipomatosis, present in some lesions, has been reported to be due to absence of dermis or a striking underdevelopment of connective tissue with replacement by adipose tissue from herniation of subcutaneous fat through multiple areas of hypoplasia. We believe this theory to be a major error in interpretation of the microscopic findings. We have had the unique experience of studying 2 patients periodically for 27-30 years and 2 additional patients for a shorter time. Biopsy specimens were removed at intervals for analysis from the same or similar lesions (43 specimens) from these 4 individuals. Our evidence strongly supports the concept that the cutaneous defects of development involving fat cells represent heterotopic fat i.e. a fat nevus resulting from dysplasia, not hypoplasia followed by herniation of subcutaneous fat.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
0303-6987
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
16
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
237-58
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pubmed:dateRevised |
2004-11-17
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pubmed:meshHeading |
pubmed-meshheading:2592623-Adipose Tissue,
pubmed-meshheading:2592623-Adult,
pubmed-meshheading:2592623-Atrophy,
pubmed-meshheading:2592623-Child,
pubmed-meshheading:2592623-Ectodermal Dysplasia,
pubmed-meshheading:2592623-Female,
pubmed-meshheading:2592623-Focal Dermal Hypoplasia,
pubmed-meshheading:2592623-Humans,
pubmed-meshheading:2592623-Infant,
pubmed-meshheading:2592623-Lipoma,
pubmed-meshheading:2592623-Papilloma,
pubmed-meshheading:2592623-Skin,
pubmed-meshheading:2592623-Skin Abnormalities,
pubmed-meshheading:2592623-Skin Neoplasms,
pubmed-meshheading:2592623-Syndrome
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pubmed:year |
1989
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pubmed:articleTitle |
Cutaneous defects of focal dermal hypoplasia: an ectomesodermal dysplasia syndrome.
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pubmed:affiliation |
Department of Dermatology, University of Texas Southwest Medical Center, Dallas.
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pubmed:publicationType |
Journal Article,
Case Reports
|