Pseudoxanthoma elasticum (PXE) is a relatively rare disease in which there is progressive mineralization and fragmentation of elastic fibers.
The skin, eye, blood vessels and heart contain elastic fibers and are consequently affected in PXE.
PXE is also known as Grönblad-Strandberg syndrome, named after a Swedish eye doctor and a Swedish skin doctor respectively who described the disease during the first half of the 20th century.
In PXE one may find yellow raised skin lesions that resemble plucked chicken skin in appearance. These lesions are located particularly in the neck, armpit, groin, elbow, knee and umbilical region.
In the eye one may see a peau d’orange (orange peel) appearance.
Goral V et al. Pseudoxantoma elasticum, as a repetitive upper gastrointestinal hemorrhage cause in a pregnant woman. World J Gastroenterol, 2007 13(28):3897-9. Go to reference
Plucked chicken skin appearance of neck