October 2nd, 2012
The myths of two of our favorite “monsters” may not be just the stuff of active imaginations and Hollywood hyperbole – could their origins rest with science and medicine?
When we think of modern day werewolves and vampires, of the former, we think of hairy, wolf-like, blood-thirsty beasts, snarling and frothing at the mouth, that transform from their human shape on the light of the full moon to attack humans. When we think of vampires, we think of pale, cloaked human-like figures with sharp canine teeth. As sunlight is harmful to them, they only come out at night to feed on the blood of humans and can transform at will into their also blood-thirsty counterparts, the vampire bat.
Many of the legends of werewolves and vampires originated from the European Middle Ages. During this time, it was common for nobility to inbreed, causing many ordinarily rare genetic diseases to be more common.
One disease in particular called Porphyria (por-fee-ree-a) involves enzyme defects of porphyrin, a protein that is part of the heme pathway. Heme is a component of hemoglobin which allows our red blood cells to hold on to oxygen. Heme is also a component of other protein groups including a set of detoxification proteins in the liver called the cytochrome p450 family. There are seven different types of Porphyria, and are classified under hepatic or erythropoeitic, depending on whether the defect occurs in liver or red blood cell porphyrins. Porphyrias have been detected in all races, in multiple ethnic groups on every continent.
In the more severe and rarer form of the Porphyrias, the symptoms start from infancy and include severe light sensitivity causing skin blistering, ulcerations, and tissue mutilation and excess facial and body hair (either coarse or fine). The teeth and even nails can become red in color from excess porphyrin deposition. Because of recession of the gum line and tautness of the skin of the lips, the teeth appear larger. Also, due to defects in porphyrin metabolism and because the red blood cells could break open (hemolytic anemia), the urine could turn brown, purple, or red and the spleen would enlarge. From liver disease, some sufferers could get jaundiced, giving their skin a yellow color from excess bilirubin. In addition, certain victims could get a rapid heartbeat with cardiac arrhythmias and high blood pressure, severe abdominal pain, and neurological and psychiatric disorders such as “lunacy,” seizures, and hallucinations.
Hmm, is this sounding familiar? Essentially, someone with severe Porphyria could only go outdoors at night. They could have extra hair making them appear animal-like. Also, they could have fang-like red teeth as if they just drank blood and red nails as if they ripped into flesh. They would behave crazily and aggressively due to their severe abdominal pain and neuropsychiatric symptoms. Though it has never been scientifically substantiated, it would not much of a leap of faith to think this may be the basis for vampire and werewolf lore.
Just to finish the story, certain medications, alcohol, hormone fluctuations, starvation, and infections would greatly worsen the symptoms. The treatment to this day, depending on whether the enzyme defect if based in liver or red blood cell enzymes, includes strict avoidance of sun and use of broad-spectrum sunscreens, certain blood product infusions OR taking blood away (phlebotomy), maintaining high carbohydrate diets, elimination of the previously mentioned triggers, and seizure and pain medications.
If it is hard for us in the modern era to believe that one disease can cause so many unusual characteristics, it is easy to envision how the active imagination of a Middle Ages mind could embellish the stories of these poor human sufferers to make them evil and ungodly.
Once again, truth may be stranger than fiction. Have a happy and safe Halloween!
Roopal Bhatt, MD is a dermatologist in the Four Points Area. To reach her for questions on this topic or others, please e-mail her at contact@fourpointsdermatology.com
