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Biochemical Analyses of Macromolecular Matrix Components in Patients with Pseudoxanthoma Elasticum
Author(s) -
Sakuraoka Koichi,
Tajima Shingo,
Nishikawa Takeji,
Seyama Yoshiyuki
Publication year - 1994
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1994.tb01422.x
Subject(s) - pseudoxanthoma elasticum , elastin , hydroxyproline , glycosaminoglycan , dermatan sulfate , dermis , hyaluronic acid , chemistry , pathology , anatomy , biochemistry , medicine , heparan sulfate
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.