Although pseudoxanthoma elasticum with angioid streaks was first described as a syndrome by Groenblad and Strandberg,1 the symptoms and signs were recognized and known by others before them. In 1889 Doyne2 reported changes of the retina similar to those described later as angioid streaks. In 1892 Pflange3 and Knapp4 described such changes as angioid streaks of the retina. The first comprehensive description of the characteristic cutaneous lesions was that by Darier5 in 1896. He described both the clinical and the histologic changes of the skin under the name of pseudoxanthoma elasticum. In 1904 Werther6 called attention to the fact that pseudoxanthoma elasticum was a familial disease. Sugg and Stetson7 reported the case of two sisters with pseudoxanthoma elasticum, angioid streaks and diabetes. Verhoeff8 reported angioid streaks in a case of osteitis deformans. In his investigation of 22 cases of osteitis deformans, Terry9