Takata Tomoya, Ikeda Mitsunori, Kodama Hajime, Kitagawa Nobuko
Department of Dermatology, Kochi Medical School, Kochi, Japan.
Pediatr Dermatol. 2007 Jul-Aug;24(4):424-5. doi: 10.1111/j.1525-1470.2007.00467.x.
Pseudoxanthoma elasticum is an inherited systemic disorder of connective tissue characterized by fragmentation of elastic fibers and calcification in cutaneous, ocular, and cardiovascular systems. Mutation in the ATP-binding cassette subfamily C member 6 gene has recently been found in people with pseudoxanthoma elasticum. However, the precise mechanisms of elastic fiber fragmentation and calcification remain obscure. Recently, it has been reported that mild chronic oxidative stress affects pseudoxanthoma elasticum fibroblasts. This suggests that reactive oxygen scavengers might improve this disorder.
