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Stories of vampires date back thousands of years. Our modern concept stems from Bram Stoker's quirky classic Dracula and Hollywood's Bela Lugosi-the romantic, sexually charged, blood-sucking outcast with a fatal susceptibility to sunlight and an abhorrence of garlic and crosses. In contrast, vampires of folklore cut a pathetic figure and were also known as the undead. In searching for some underlying truth in vampire stories, researchers have speculated that the tales may have been inspired by real people who suffered from a rare blood disease, porphyria. And in seeking treatments for this disorder, scientists have stumbled on a new way to attack other, more common serious ills.
Porphyria is actually a collection of related diseases in which pigments called porphyrins accumulate in the skin, bones and teeth. Many porphyrins are benign in the dark but are transformed by sunlight into caustic, flesh-eating toxins. Without treatment, the worst forms of the disease (such as congenital erythropoietic porphyria) can be grotesque, ultimately exacting the kind of hideous disfigurement one might expect of the undead. The victims' ears and nose get eaten away. Their lips and gums erode to reveal red, fanglike teeth. Their skin acquires a patchwork of scars, dense pigmentation and deathly pale hues, reflecting underlying anemia. Because anemia can be treated with blood transfusions, some historians speculate that in the dark ages people with porphyria might have tried drinking blood as a folk remedy. Whatever the truth of this claim, those with congenital erythropoietic porphyria would certainly have learned not to venture outside during the day. They might have learned to avoid garlic, too, for some chemicals in garlic are thought to exacerbate the symptoms of the disease porphyria, turning a mild attack into an agonizing reaction.