Sunday, June 22, 2008

Proteus Syndrome: Beyond the Elephant Man

Proteus syndrome, also known as gigantism, is best know because it was the condition suffered by Joseph Merrick, the subject of the book, play, and film "The Elephant Man." Proteus syndrome causes the overgrowth of skin on the body and also deformed bone growth. The result can be club feet and hands (which is where the "Elephant Man" identification came from), as well as on the head and other areas of the body.

Proteus syndrome is a congenital disorder that can involve many different body systems, and besides the extreme disfiguration it causes, it presents some very serious health risks. Proteus syndrome is, fortunately, quite rare: it's estimated that only 100 to 200 people worldwide have it (or have been diagnosed with it, anyway).

You can find a good layman's overview of Proteus syndrome at the eMedicine.com Web site. The Proteus Syndrome Foundation also features a lot of information, including definitions, symptoms, criteria for diagnosing Proteus syndrome, and a glossary of terms used.

For the more scientifically oriented reader, you'll find details and further reading at the Web site of the National Center for Biotechnology Information.

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