Biochemical analyses of macromolecular matrix components in patients with pseudoxanthoma elasticum

Two cases of pseudoxanthoma elasticum (PXE) were reported. Biochemical studies of collagen, glycosaminoglycan, and elastin content were performed using skin specimens from affected lesions. Hydroxyproline content was reduced approximately 46% and 24% in cases 1 and 2, respectively. The reduction in...

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Bibliographic Details
Published inJournal of dermatology Vol. 21; no. 2; p. 98
Main Authors Sakuraoka, K, Tajima, S, Nishikawa, T, Seyama, Y
Format Journal Article
LanguageEnglish
Published England 01.02.1994
Subjects
Adult
Collagen - analysis
Elastin - analysis
Extracellular Matrix - chemistry
Female
Glycosaminoglycans - analysis
Humans
Hydroxyproline - analysis
Middle Aged
Pseudoxanthoma Elasticum - metabolism
Pseudoxanthoma Elasticum - pathology
Skin - chemistry
Skin - pathology
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Summary:Two cases of pseudoxanthoma elasticum (PXE) were reported. Biochemical studies of collagen, glycosaminoglycan, and elastin content were performed using skin specimens from affected lesions. Hydroxyproline content was reduced approximately 46% and 24% in cases 1 and 2, respectively. The reduction in collagen content resulted from the reduction in the lower dermis, not from that in the upper dermis. Glycosaminoglycan content in PXE was also decreased, with a slight increase in the ratio of hyaluronic acid to dermatan sulfate. Isodesmosine content in PXE was increased 3.2- and 2.2-fold in cases 1 and 2, respectively. These results suggest that the major and primary biochemical changes in PXE are an increase in elastin content.
ISSN:0385-2407
DOI:10.1111/j.1346-8138.1994.tb01422.x