Bier's white spots associated with scleroderma renal crisis

Summary Bier's spots are asymptomatic and permanent white macules, standing on a cyanotic background. These spots are associated with venous stasis and usually appear in physiological conditions. We report the case of a 47-year-old woman with a 2-year history of systemic scleroderma, who developed Bier's white spots associated with a vascular and renal crisis. Interestingly, these spots appeared with the crisis, and disappeared with the treatment and resolution of the crisis. They are probably a result of anatomical and functional damage to the small vessels of the skin. The pathophysiology in the skin is probably the same as that which happens in renal vessels during scleroderma renal crisis.