What is Proteus?

Proteus Syndrome is a condition which involves atypical growth of the bones, skin, head and a variety of other symptoms. Proteus syndrome was first identified by Michael Cohen Jr., DMD, PhD., in 1979. The name comes from the greek God Proteus who used to change his shape or form to avoid capture. it is a very rare, variable and progressive condition, affecting more males than females. The cause of Proteus syndrome is unknown. The syndrome became more widely known when it was determined that Joseph Merrick (the patient depicted in the play and movie "The Elephant Man") had severe Proteus syndrome rather than Neurofibromatosis as had been suggested previously.

What are the signs of Proteus Syndrome

  • Overgrowth, asymmetry and gigantism of the limbs
  • Increased size of an organ, or the body, or bones (Hypertrophy)
  • Raised rough skin (verrucous epidermal naevi)
  • Deep lines and overgrowth of soft tissue on the soles of the feet (cerebriform connective tissue nevus)
  • Patches of overgrown blood or lymphatic vessels (vascular malformations)
  • Local overgrowth of fat (lipomas)or undergrowth of fat
  • Various tumours are more common in patients with Proteus syndrome, but most are benign
  • Deep vein thromboses (blood clots) and the lodging of these blood clots in the lungs. This can be life threatening.

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