[Netka]

Автор оригинала: Alise

А ты "сбрось" ее сюда в открытом виде (если статья небольшая) или в виде приложения (если объемная) - вдруг кто да переведет...

Всегда пожалуйста:
Has anyone ever wondered what medical condition created the Phantom? Susan Kay simply made her villain a victim of a congenital physical deformity present at birth. This was not the case of the Phantom in Leroux however.

When Gaston Leroux first explored the depths of the Opera House, he came up with the idea for a fascinating villain. He remembered the prisoners held during the French Commune and in his early notes he imagined a child who had been forgotten when the prisoners were freed. The boy eventually went mad and made the underworld his home. Leroux imagined the child becoming an architectural and musical genius who designed secret rooms in the depths of the Opera House.

Leroux began to formulate what type of person could survive in the eerie underworld. Because of his years of practical journalism, Leroux wanted a character within the realms of possibility, not a mythical monster.

Leroux began searching the medical reference books of his large library for a malady which could render his musical genius insane whithout affecting his intellect. He began noting what the physical effects would be on a body deprived of sunlight, clean water and sanitary conditions over a period of years.

In 1909, he obtained copies of recently published research papers from London concerning Joseph Merrick, also known as The Elephant Man, who suffered from von Recklinghausen's neurofibromatosis. Although Leroux considered Joseph Merrick's deformities too great and too fragile for his character who needed to be able to be athletic, draw and play musical instruments, he was intrigued with certain aspects of Merrick's story.

One of the transcripts told how Merrick almost died when a woman visitor politely shook hands with him. It was the first time that a woman had ever smiled at him and shaken his hand. The overwhelming emotion taxed his weakened state and placed him in shock. He almost died from the joy of the moment. This event gave considerable insight to Leroux as to how kindness can affect one whose life has been scarred with abuse and rejection due to physical appearance.

That same year a physician friend visited Leroux and gave him the answer for his quest for a disorder that would fit the physical and mental profile of his mad villain. A German medical journal, Strahlentherapie, provided Leroux photographs plus signs and symptoms of the disease. The answer, a disease know as Porphyria Cutania Tarda (PCT), produces tragic symptoms in those afflicted.

(I apologize in advance to Le Phorum members for getting a little technical here, but I just want to give a complete description).

The porphyrias represent abnormalities in the pathway for heme synthesis. Porphyria Cutania Tarda is the most common form and results from a deficiency of hepatic uroporphyrinogen decarboxylase activity. It occurs when the heme biosynthetic pathway is compromised by an exogenous agent. The disorder becomes progressively worse by exposure to sunlight.

Prolonged contact to sunlight in a victim of PCT causes a severe photosensitive reaction in the skin and soft tissues of exposed areas. This results in swelling and a red, blistery rash. There is a tendency for skin lesions to ulcerate and erode. These ulcers may attack cartilage and bone with resultant loss of structure. Significant scarring also occurs. Over time, structures such as ears, nose, eyelids and lips undergo progressive mutilation. There would be patchy loss of hair. Significant loss of skeletal muscle bulk would render the victim almost skeletal. Liver disease can occur in PCT and subsequent jaundice produces a pale, yellow skin. Teeth may have a reddish-brown discoloration due to the deposition of porphorins. Nervous manifestations include mental disorders ranging from mild hysteria to manic-depressive psychoses and delirium.

As you can see, PCT provided the perfect explanation for the physical and mental deformities of Leroux's Phantom, whose madness would also be his genius. His nervous disorder would be a gradual mental breakdown. Normal thought processses and knowledge would remain, while reality would gradually erode.

Leroux now had his practical explanation for both the pitiful physical deformities and mental madness of his genius villain. He subsequently wrote his book and the rest is history!!!