Introduction to Proteus Syndrome
Proteus Syndrome - condition which involves atypical growth of the bones, skin, head and a variety of other symptoms.
Name of Disorder
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In 1983 a German Pediatrician, Hans-Rudolf Wiedemann, named it proteus syndrome, for the Greek God Proteus “the polymorphous” presumably because of the variable manifestation in the four unrelated boys first identified with the syndrome.
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Other Names:
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Elattoproteus syndrome
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Gigantism
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Encephalocraniocutaneous lipomatosis
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Elephant man syndrome
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This disease was first identified by Michael Cohen Jr., DMD, PhD., in 1979.
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Proteus syndrome is a progressive condition. This is when children are usually born without any obvious deformities, but as they age, tumours as well as skin and bone growths appear.
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The classification of Proteus syndrome is not yet confirmed, but many sources classify Proteus syndrome to be a type of nevus syndrome. Due to the mosaic distribution of lesions, it is hypothesized that the disorder is indeed an example of genetic mosiacism.
