Three-dimensional architecture of altered dermal elastic fibers in pseudoxanthoma elasticum: scanning electron microscopic studies.

The three-dimensional architecture of the altered dermal elastic fibers of patients with pseudoxanthoma elasticum has been demonstrated using a scanning electron microscope. Each of the altered elastic fibers could be divided into three parts: (i) a normal-looking, slender part which is composed of thinner fibrils, (ii) a thickened, tortuous part covered with an amorphous substance, and (iii) a markedly damaged part where calcium is exposed to the surface of the fibers. The presence of calcium and phosphorus in the altered elastic fibers has been proved by an electron x-ray microanalysis.