Proteus Syndrome
By: Caleb Bentley
14 July 2011
Disclaimer: This report is not intended to provide medical advice, diagnosis, or treat any diseases. It is simply for informational and educational purposes to help further knowledge in the subject area. Please enjoy…
Named after a Greek sea god who could change his shape, Proteus Syndrome, or Proteus, is a disease characterized by abnormal growth of body parts (1). It is a rare congenital disorder (approximately 200 confirmed cases worldwide), involving the abnormal growth of the bones, skin, or fatty tissues in those affected by this disease, and Hemihypertrophy - overgrowth of one side of the face, body, or limbs. Though it is unclear as to what causes Proteus, some research suggests that a gene mutation (either chromosome 10 or 16) is the cause of this disorder, but nothing has yet been confirmed.
Each case has extreme variations, ranging from mild, over growth, of fingers and hands, toes and feet; to severe, gigantism of limbs or significant portions of the body (2). This is known as hypertrophy. Due to the variations, diagnosis can be very difficult. Proteus is not considered to be a fatal disease, rather complication early in life are attributed to Proteus are the primary cause of death in most of those who are affected by it.
Although Proteus can visually appear similar to Elephantiasis, they are two different diseases (3, 4). Elephantiasis, or Lymphatic filariasis, occurs when a mosquito carrying a parasitic worm called Brugia malayi, takes a human meal and infects the human with the parasite. The parasite invades the lymphatic system, and, for lack of a better term, stops at a specific spot and begins breeding, thus clogging the vessel. The worms blocking fluids from leaving the limb, causes excessive fluids to accumulate in the limbs surrounding tissues, which causes the swelling associated with the disease. Kind of like in cartoons when the fire hose gets clogged up and the segment of the hose between the knot and the water source gets bigger and bigger.
(5) Joseph Merrick, A.K.A. "the elephant man", is probably the most famous individual, who is believed, to have this disease. Born on August 5, 1862, Mr. Merrick skin began to darken and rough growths began to appear on his body. Unable to find work or due manual labor, due to the growths on his hands and face, Mr. Merrick began working as a side show in 1884 to earn money. A few months after beginning his “work”, Dr. Frederick Treves visited the show where Mr. Merrick was on display. Dr. Treves asks to present Mr. Merrick to the London Pathological Society, to which he agrees. In June 1886, Mr. Merrick is admitted to the London Hospital for treatment, where he lives until his death on April 11, 1890. He died from asphyxiation in his sleep, possibly due to the weight of his own head. With Mr. Merrick’s approval in life, his body was cast in plaster post-mortem and his skeleton in still on display at the London Hospital for scientific and medical research.
As of this report (July 2011), there is neither treatment nor cure for Proteus. Surgery can cut away some of the infected tissue to lessen the appearance or prolong life, but that is about it.
This report is pretty short due to the lack of research into this disease. Most of the sources I found said the same things: it’s most likely caused by a genetic mutation, there are wide variations in the severity of this disease, Joseph Merrick is possibly the most well known person who was affected by this disease, and there is no known cure or treatment.
Thank you for taking the time to read what I had to say. Please, feel free to leave any comments below and let me know what you though. As always, please be respectful of others opinions, even if they do not agree with your own.
Word Sited:
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| Gigantism/ Proteus Syndrome of the feet and toes |
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| Possible Proteus of the arms |
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| Joseph Merrick 1 |
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| Joseph Merrick 2 |
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| Joseph Merrick's skeleton from the London Hospital |
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| Elephantiasis life cycle |
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| Elephantiasis of the legs 1 |
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| Stage 3 Elephantiasis |
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| Elephantiasis 2 |
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