Proteus syndrome

GC: n

S: (last access: 30 July 2014); EncBrit – (last access: 30 July 2014); DORLAND p. 1845.

N: 1. A disturbance of cell growth including benign tumors under the skin, overgrowth of the body, often more on one side than the other (hemihypertrophy), and overgrowth of fingers (macrodactyly). The syndrome is named after the Greek god Proteus the polymorphous who could change his appearance.
2. Proteus syndrome is a rare condition characterized by overgrowth of the bones, skin, and other tissues. Organs and tissues affected by the disease grow out of proportion to the rest of the body. The overgrowth is usually asymmetric, which means it affects the right and left sides of the body differently. Newborns with Proteus syndrome have few or no signs of the condition. Overgrowth becomes apparent between the ages of 6 and 18 months and gets more severe with age.
3. In people with Proteus syndrome, the pattern of overgrowth varies greatly but can affect almost any part of the body. Bones in the limbs, skull, and spine are often affected. The condition can also cause a variety of skin growths, particularly a thick, raised, and deeply grooved lesion known as a cerebriform connective tissue nevus. This type of skin growth usually occurs on the soles of the feet and is hardly ever seen in conditions other than Proteus syndrome. Blood vessels (vascular tissue) and fat (adipose tissue) can also grow abnormally in Proteus syndrome.
4. Cultural Interrelation: Elephant Man (1980) from David Lynch. The disorder from which Merrick suffered was long thought to be an extremely severe case of neurofibromatosis, but his deformities were probably the result of an extremely rare disease known as Proteus syndrome.

S: 1. (last access: 30 July 2014). 2 & 3. (last access: 30 July 2014). 4. EncBrit – (last access: 30 July 2014); FCB.


CR: elephantiasis, neurofibromatosis.