The scheme of the relationship between angioid streaks (AS) and pseudoxanthoma elasticum (PXE). The prevalence of AS and clinically manifested PXE were 6.5 and 0.83 per 100 000 persons, respectively. Among the study population, there were 363 cases of overlap between AS (8196 patients) and clinically-manifested PXE (1050 patients). Of those with overlapping conditions, 63.9% were initially diagnosed with AS, 25.3% with PXE, and the remaining 10.7% were diagnosed with both conditions simultaneously. In previous reports, skin biopsies were performed on 44 consecutive cases of AS, resulting in a diagnosis of PXE in > 90% of cases. Given these findings, the present study observed a remarkably low incidence of overlapping AS and PXE cases. These results suggest that in a substantial number of AS patients, the comorbidity of PXE may be overlooked or PXE may remain subclinical, warranting further investigation.