Document Detail

Light and electron microscopy of pseudoxanthoma elasticum
MedLine Citation:
PMID:  4040128     Owner:  NLM     Status:  MEDLINE    
Five patients with recessive pseudoxanthoma elasticum (PXE-R) and four with dominant transmission of the disease (PXE-D) belonging to the same family were studied by light and electron microscopy. In PXE-R, calcification in the elastic fibres causes their enlargement, excavation and fragmentation. On the other hand, in PXE-D irregularly shaped and unevenly lined elastic strands may form an anastomotic wickerwork intimately intermingled with the collagenous texture. In other places, elastic bundles seem to be composed of very tiny elements. Both aspects represent a dysplasia of the elastic tissue. Independently of the mode of inheritance, a variable proportion of enlarged collagen fibrils exhibit a "flower-like" structure. Additionally, in PXE-D alone a peculiar aggregation of small and large collagen fibrils is observed. Granulofilamentous material mixed with tiny collagen fibrils is found in both groups of patients. On the basis of our observations, PXE-R and PXE-D may be identified by light and electron microscopy.
A Vogel; U W Schnyder; S B Stutz
Publication Detail:
Type:  English Abstract; Journal Article    
Journal Detail:
Title:  Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete     Volume:  36     ISSN:  0017-8470     ISO Abbreviation:  Hautarzt     Publication Date:  1985 May 
Date Detail:
Created Date:  1985-08-12     Completed Date:  1985-08-12     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372755     Medline TA:  Hautarzt     Country:  GERMANY, WEST    
Other Details:
Languages:  ger     Pagination:  269-73     Citation Subset:  IM    
Vernacular Title:
Zur Licht- und Elektronenmikroskopie des Pseudoxanthoma elasticum (PXE).
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MeSH Terms
Cytoskeleton / ultrastructure
Elastic Tissue / ultrastructure
Genes, Dominant
Genes, Recessive
Microscopy, Electron
Pseudoxanthoma Elasticum / genetics,  pathology*
Skin / pathology,  ultrastructure
Reg. No./Substance:

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