This is because of a condition called methemoglobinemia, which causes methemoglobin levels in the red blood cells to rise above 1 percent. It turns the skin blue, the lips purple, and the blood a chocolate brown. Methemoglobinemia can be triggered by exposure to particular chemicals (benzocaine and xylocaine, for example), but in this case, it was inherited and the product of a faulty gene that most probably caused a deficiency in an enzyme called cytochrome-b5 methemoglobin reductase.
Fortunately for the Fugates and their kin, there are no physical health problems associated with their blue skin. In fact, most survived well into their eighties and nineties.
That isn't to say it wasn't a deep source of shame and psychological trauma. The family were embarrassed and discriminated against by their local community because of their skin color. This caused them to seek greater social isolation, which somewhat ironically, exacerbated the problem. This is because methemoglobinemia is, in almost all cases, a product of inbreeding. [...]
After a few medical tests to make sure it wasn't heart disease, the doctor and the nurse created a family tree. He suspected methemoglobinemia but couldn't be sure what was causing it. There were several suspects, including abnormal hemoglobin formation and excessive vitamin K consumption, but blood tests eventually revealed the true culprit: The blue Fugates lacked the enzyme diaphorase.
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