Pseudoxanthoma elasticum and angioid streaks: A review of 106 cases

Abstract

The records of 106 patients given the diagnosis of pseudoxanthoma elasticum, angioid streaks, or both (Grönblad-Strandberg syndrome) at the Mayo Clinic from 1931 through 1958 were reviewed. Seventy-four patients had pseudoxanthoma elasticum, and sixty-three of these seventy-four patients (85 per cent) had associated angioid streaks in the ocular fundi. Ninety-five patients had angioid streaks, and thirty-two of these patients (34 per cent) had no clinical evidence of associated pseudoxanthoma elasticum; two patients with angioid streaks had associated osteitis deformans. The greatest cause of morbidity in this series was loss of central vision which occurred in sixty-nine of the ninety-five patients (73 per cent) who had angioid streaks of the ocular fundi; forty-nine (51 per cent) of these patients experienced visual problems before the sixth decade of life. A small number of female patients with pseudoxanthoma elasticum complained of the unsightliness of the skin lesions. The characteristic clinical and histologic findings in the skin and the ophthalmoscopic findings are discussed. Sixteen per cent of the seventy-four patients with pseudoxanthoma elasticum had clinical evidence of occlusive peripheral arterial disease at a younger mean age than would ordinarily be expected, and 26 per cent had clinical evidence of occlusive peripheral, coronary or cerebral arterial disease. The incidence of hypertension in these patients appears to be relatively high. Ten patients with pseudoxanthoma elasticum and two with angioid streaks alone had episodes of gastrointestinal bleeding, some of which were serious. In three cases histologic examination of surgically excised tissue revealed degenerative changes of elastic tissue in the internal elastic lamina of certain arteries. Iron was present in the areas of elastic tissue degeneration, a finding which, to our knowledge, has not been previously reported in the arteries of patients with pseudoxanthoma elasticum.