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hypertrichosis - The Curse of the Hair

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  • hypertrichosis - The Curse of the Hair


    The Curse of the Hair

    By Guenther Stockinger
    December 31, 2004
    Translated from the German by Christopher Sultan

    Wolf people suffer from one of the world's rarest genetic diseases: Their entire bodies are covered by a thick coat of fur. Only about 50 cases of the disorder have been documented since the Middle Ages. The recent discovery of a case of the disease in Germany has sparked new interest in hypertrichosis.Genetic defect

    A genetic defect causes the hair growth cycle in victims of this rare disease to run amok. The follicles from which the body hair grows are apparently incapable of switching from the growth phase to the dormant phase, which normally ends in the new hair falling out and the cycle beginning again.

    This "curse of the hair" could be caused by a primeval gene stemming from our animal ancestors. Scientists believe that many of these primeval genes still lie dormant in the human genome, and that they have simply been switched off during the course of evolution. A mutation in the victims of hypertrichosis could have led to such a gene being awakened from its million-year slumber.

    Doctors and biologists are familiar with a wide variety of such phenomena, known as atavisms. In rare instances, for example, children are born with additional nipples, which, like the rows of nipples normally found on mammals, run from the armpit to the pelvic region. Children are also occasionally born with a small tail-like protrusion at the base of the spine.

    However, the atavism theory is contradicted by the fact that people suffering from hypertrichosis also have dense hair growth in areas - the nose and area around the eyes - that are largely devoid of fur in other primates like chimpanzees and gorillas. Experts such as Baumeister also point out that metabolic disorders can cause hypertrichosis on the entire body. The same phenomenon can also be acquired later in life in conjunction with certain diseases, such as multiple sclerosis, or as a side-effect of some drugs.

    Both men and women affected by the mutation pass on the defective gene to 50 percent of their offspring. The line ends in family members who are born with normal hair growth.

    There are various types of hypertrichosis, depending on the human hair type affected by the genetic defect. Some victims experience uncontrolled growth of the fine, non-pigmented lanugo hair, which covers the entire body of the fetus, including its face, beginning in the fifth month of pregnancy. Fetuses normally lose this hair suit between the seventh and eighth months of pregnancy. In some victims of hypertrichosis, however, this natural process takes years and often ends only in adulthood.

    However, uncontrolled hair growth on the body can also affect fully pigmented terminal hair. The best-known example of this especially disfiguring form of hypertrichosis was Julia Pastrana, a Mexican Indian born in 1834, whose entire body - except the soles of her feet and her palms -- was covered with long, thick black hair. From 1854 on, Pastrana, along with her greedy husband, impresario Theodore Lent, traveled the world from one circus to another.

    Gaping audiences in London, Berlin and Moscow were horrified by the sight of the "ape woman." But Pastrana was gentle and intelligent, could read and write, played the guitar and sang in a beautiful mezzo-soprano voice. She died at the age of 26, only five days after giving birth to a child covered entirely with hair. Pastrana had desperately hoped that her child would be born without the defect.

    This form of hypertrichosis has also affected five generations of a Mexican family in which children were consistently born with darkly pigmented hair covering their entire bodies. Some now work as trampoline acrobats in circuses. Others shave their faces several times a day or remain as far away from other people as possible.

    Neuropediatrician Baumeister believes that the family of Pedro Gonzalez, the famous servant of Henry II, owed his animal-like appearance to a third hair type known as vellus. This type of hair grows in all human beings as a soft, silky and often pigmented down in areas of the body where no terminal hair is produced. In this third form of hypertrichosis, however, this fine downy hair grows uncontrollably. In hypertrichosis victims, vellus, which is normally no longer than about a centimeter, can proliferate to lengths of up to 30 centimeters (about twelve inches) if left unchecked. Baumeister has so far found descriptions of nine such cases in the scientific literature. They include Polish-born Stephan Bibrowsky, whose mane-like facial hair gave him the appearance of a lion and who appeared as "Lionel the Lion Man" in circuses and vaudeville shows in the early 1900s.

    A boy suffering from the same form of hypertrichosis was born in Germany in 1958. He has since disappeared, and the only remaining existence is an early photograph in the professional literature. It is an image of a boy who appears to be about six years old. He sits with his hands on his knees and gazes sadly into the camera. His face is so absurdly buried under a proliferating mane of hair as to give him the appearance of an old man ravaged by life.

    Exceptional virility?

    Men seem to adjust to this genetic disorder more easily than women, as evidenced by the history of the Gonzalez family. Author Zapperi believes that "their excessive hair may actually have heightened the appeal of the males in the family, because it created the impression of exceptional virility."

    Don Pedro, as the "furred one" liked to call himself, defiantly alluding to his ancestors, a family of chieftains in the Canary Islands, married a beautiful French woman with whom he raised several children, including three daughters and two sons who shared their father's animal-like appearance.

    After the death of French King Henry II, the members of the Gonzalez family were given to other royal families, where they were gaped at and painted. Four large paintings of the Gonzalez family still hang in Ambras Castle near Innsbruck, Austria. The faces of the subjects, offset by white ruffled collars, look like an evil trick of nature. Their bearing gives them the appearance of elegant human beings, but the hair covering their faces suggests proximity to the animal kingdom.

    After years of being gawked at, Enrico, the eldest son of Don Pedro, managed to trick his master into allowing the family to live quietly in the small village of Capodimonte on Lake Bolsena in Italy. He convinced his master, Cardinal Odoardo Farnese, that he and his family, as wild creatures, were magically drawn to a life in nature. Farnese, a religious man from Rome, was ultimately unable to resist Enrico's convincing but tall tale of the powers of animal instinct.

    In the remote village, inhabited by only a few farmers and fishermen, Enrico brought together the widely dispersed members of his extended family, married healthy women several times, and managed to achieve modest wealth as a businessman. His father, Don Pedro, spent the last few years of his life in Enrico's idyllic village, and died peacefully at the advanced age of about 80.

    But the fate of the hirsute Gonzalez daughters is more reminiscent of the suffering of "ape woman" Pastrana. Like their father, they lived long lives, but theirs was a hidden existence.

    Picture: "Wolfman" Fajardo Aceves Jesus Manuel of Mexico (source: DPA),00.html
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  • #2
    Re: THE WOLF PEOPLE - The Curse of the Hair

    'Werewolf boy' begs for cure to rare condition


    An 11-year-old boy whose face and body are covered with hair is baffling medical experts.'Werewolf boy' Pruthviraj Patil in Pictures
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    • #3
      Re: hypertrichosis - The Curse of the Hair

      Oh Allah (swt), Oh Rahim, Oh Rahman, Save us from such calamities. Ameen.
      Salam! :)



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