Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
1995-10-10
pubmed:abstractText
To elucidate the mechanisms involved in the development of cutaneous fibrosis in scleredema adultorum, we studied a patient with long-standing scleredema who had no history of diabetes mellitus or preceding febrile illness. Histological examination of a biopsy specimen from involved forearm skin demonstrated marked thickening of the dermis and accumulation of mucin between collagen bundles. Increased levels of type I collagen mRNA, as evidenced by positive in situ hybridization signals with an alpha 1(I) procollagen cDNA were found in numerous fibroblasts throughout the dermis. The expression of several genes coding for proteins involved in the maintenance of connective tissue was examined by determining in vitro protein production and mRNA levels in fibroblasts from the affected skin. Total protein production, glucosamine incorporation and collagen synthesis, were elevated by 44-97% in scleredema fibroblasts, compared with fibroblasts from two healthy individuals. Levels of mRNAs for alpha 1(I) and alpha 1(III) procollagens and fibronectin were elevated in scleredema fibroblasts, whereas mRNA levels for the tissue inhibitor of metalloproteinase were unaltered compared with control cultures. The results suggest that fibroblasts from the involved skin in non-diabetic patients with scleredema may exhibit a biosynthetically activated phenotype, which persists for several years. These alterations are likely to be involved in the development of the cutaneous induration and thickening which is characteristic of this disease.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
0007-0963
pubmed:author
pubmed:issnType
Print
pubmed:volume
132
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
992-9
pubmed:dateRevised
2008-11-21
pubmed:meshHeading
pubmed:year
1995
pubmed:articleTitle
Scleredema adultorum: case report and demonstration of abnormal expression of extracellular matrix genes in skin fibroblasts in vivo and in vitro.
pubmed:affiliation
Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA 19107, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Case Reports