Porphyria (sometimes called Vampire Syndrome) is a medical term which refers to a group of rare metabolic disorders which are genetic conditions which are characterized by an overproduction of organic compounds called porphyrins. The overproduction of porphyrins results from a deficiency in heme (pigment in red blood cells). The body tries to compensate for the heme deficiency by producing more porphyrins than the body needs. There are two major types of porphyria and several subtypes; each type of porphyria is caused by an overproduction of a certain type of porphyrin. Porphyria isn’t just one disease, but it is a group of genetic disorders sometimes called the porphyrias.
Types of Porphyria
The two main types of porphyria are acute porphyria and cutaneous porphyria. Each type of porphyria contains subtypes that are characterized by a deficiency of a certain type of enzyme that is needed for the body to create heme. The subtypes and deficient enzymes are too numerous to mention in this article; therefore, you can read the chart to see the types, subtypes and the deficient enzymes here.
Famous people in history who may have had porphyria
It is believed that the group of illnesses referred to as the porphyrias started the legendary vampires. It is believed that porphyria originated from intermarriage within European nobility. Porphyria is a relatively new medical term; in the past, it was called liver disease, blood disease, and later it was called vampire disease, which is said to have started the legend of Dracula the vampire.
It is believed that Mary Queen of Scots, George III, Queen Anne (Great Britain), Fredrick the Great (Germany), George IV (Great Britain), Princess Charlotte (daughter of George IV died at birth) are believed to have had porphyria.
Why is porphyria called vampire syndrome?
Porphyria causes psychological problems that can lead to depression, hallucinations, and mental breakdown. Porphyria can also cause physical manifestations which can give the sufferer a frightening appearance. In history, before there was more knowledge about this rare group of conditions, the people suffering from porphyria were feared and labeled vampires.
Vampire legends and how they relate to the symptoms of porphyria
Sensitivity to light – In the vampire legends, vampires had to avoid the sun because the sun would destroy them. In actuality, people suffering from porphyria need to avoid the sun because the sun’s rays can cause discoloration and disfigurement of the skin.
If porphyria affects the eyes, the person may also suffer from photophobia.
Drinking blood – One of the symptoms of porphyria is that the urine and tears are tinged purple. It was believed that this was evidence that people drank blood. It is true that some people with porphyria did drink blood, but it was animal blood, not human.
Aggression – People suffering from porphyria often experience behavior changes. The overproduction of porphyrins has a toxic affect on the brain, which could account for behavioral changes and psychiatric manifestations.
Aversion to mirrors – The wasting away of the tissues can give the face a deformed appearance. When tissue wastes away from the mouth and gums, the person suffering from the disorder may look like he/she is growing fangs. It could be that people with this disease didn’t want to see their reflection.
Aversion to garlic – Exposure to garlic increases the discomfort from porphyria. A few hundred years ago, before porphyria was understood, many people wore garlic around their necks to protect themselves from vampire attacks.
Conclusion
Because people who have porphyria have some of the manifestations attributed to the mythological creature, many people around the world still believe in the existence of vampire in this modern day. The teeth of people with porphyria may be stained red from the buildup of porphyrins on the teeth. It is no wonder that before much was known about porphyria, that the people with the disease were feared as bloodthirsty vampires.
The diagnosis and treatment of porphyria will be discussed in a future article.
Sources:
http://www.wisegeek.com/what-is-porphyria.htm
http://digestive.niddk.nih.gov/ddiseases/pubs/porphyria/index.htm
