Phenazopyridine induced methaemoglobinaemia associated with decreased activity of erythrocyte cytochrome b5 reductase.

A 25-year-old woman taking usual doses of phenazopyridine developed her third clinically significant episode of cyanosis. Life threatening methaemoglobinaemia was documented and was treated with methylene blue. The patient and several members of her family showed decreased activities of erythrocyte NADH-cytochrome b5 reductase, predisposing them to the development of clinically significant methaemoglobinaemia when challenged with oxidant drugs.