What is blue skin disorder?
People whose blood is low in oxygen tend to have a bluish color to their skin. This condition is called cyanosis. Depending on the cause, cyanosis may develop suddenly, along with shortness of breath and other symptoms. Cyanosis that is caused by long-term heart or lung problems may develop slowly.
What can cause a family to inherit blue skin?
The Fugates, a family who lived in the hills of Kentucky, commonly known as the “Blue Fugates” or the “Blue People of Kentucky”, are notable for having been carriers of a genetic trait that led to the blood disorder methemoglobinemia, which causes the appearance of blue-tinged skin.
What is blue skin called?
Methemoglobinemia can be inherited or acquired. Acquired cases are more likely to occur and are usually the result of exposure to certain medications or chemicals such as nitrates, silver or benzocaine. Symptoms of this rare disease can include: Bluish colored skin.
Is there a cure for methemoglobinemia?
The condition is benign. There is no effective treatment for people with a congenital form who develop an acquired form. This means that they should not take drugs such as benzocaine and lidocaine. People who acquire methemoglobinemia from medications can completely recover with proper treatment.
How is methemoglobinemia treated?
Methylene blue is the primary emergency treatment for documented symptomatic methemoglobinemia. It is given in a dose of 1-2 mg/kg (up to a total of 50 mg in adults, adolescents, and older children) as a 1% solution in IV saline over 3-5 minutes.
What triggers methemoglobinemia?
The most common cause of congenital methemoglobinemia is cytochrome b5 reductase deficiency (type I b5R). This enzymatic deficiency is endemic in certain Native American tribes (Navajo and Athabaskan Alaskans). Most cases of methemoglobinemia are acquired and result from exposure to certain drugs or toxins.
What state is most inbred?
Inbreeding is more common in the following states: Washington, Oregon, Montana, South Dakota, New Mexico, Oklahoma, Arkansas, Louisiana, Indiana, Kentucky, Tennessee, Alabama, Georgia, South Carolina, North Carolina, Virginia, West Virginia, Maryland, Delaware, and Maine.
Is methemoglobinemia reversible?
Sulfhemoglobinemia should be considered in cases presenting with oxygen desaturation and cyanosis, especially if methemoglobinemia can be excluded. Unlike methemoglobinemia, which is reversible with a known antidote, methylene blue, sulfhemoglobinemia is irreversible with no known antidote.
How do you reverse methemoglobinemia?
Methylene Blue is the standard first-line antidote for methemoglobinemia. The drug acts as a cofactor for NADPH reductase and ultimately increases the rate of conversion of ferric methemoglobin to ferrous hemoglobin.
Is there a treatment for methemoglobinemia?
What causes the skin to turn blue?
Another disorder that is known to cause the skin to turn to a bluish or silver hue is argyria. This condition results from extended amounts of contact with silver compounds or the ingestion of silver salts. People who are potentially at risk of developing this condition are those:
Why do people with hemoglobin A deficiency have blue skin?
Individuals who have this disease are at risk of developing very dark, almost chocolate- colored blood or blue skin. This is because the hemoglobin that they produce is unable to carry sufficient amounts of oxygen throughout the body and oxygenate tissues.
Did you know that some people have blue skin?
Yes, it turns out, and a family living in Appalachia had the condition for generations. In their case, blue skin was caused by a rare genetic disease called methemoglobinemia.
Why do Alaskans have blue skin?
Scott, who worked in public health at the Arctic Research Center in Anchorage, had seen a recessive genetic trait among Alaskans that turned their skin blue. That suggested an inbred line that had been passed from generation to generation.