Acquired Localized Cutis Laxa due to Increased Elastin Turnover.

ABSTRACT: Cutis laxa is a rare disease characterized by abnormal skin wrinkling and laxity, due to decreased elastin synthesis or structural extracellular matrix defects. We have explored elastin metabolism in a case of adult onset cutis laxa localized to the upper body of a woman. For this purpose, we obtained skin biopsies from affected and unaffected skin areas of the patient and analyzed these with microscopy, polymerase chain reaction, western blotting and cell culture experiments. Skin from the affected area lacked elastin fibers in electron microscopy but had higher mRNA expression of elastin and total RNA. Levels of an apparent tropoelastin degradation product were higher in the affected area. Fibroblast cultures from the affected area were able to produce elastin and showed higher prolifera

SUBMITTER: Nygaard RH 

PROVIDER: S-EPMC4899661 | biostudies-literature | 2016 Jan-Apr

REPOSITORIES: biostudies-literature