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It can affect tissue from any germinal layer. Proteus syndrome is an uncommon state of abnormal growth. Proteus syndrome is a rare condition that can cause physical differences and changes over time. This name was chosen to reflect the gradual changes a person when through who was diagnosed with Proteus Syndrome. Many parents and families are frustrated by how long it can take to get a diagnosis and how hard it can be to find a doctor with experience treating this condition. The clinical and radiographic manifestations of Proteus syndrome are highly variable. For instance, one hand may be longer or wider than the other. Proteus syndrome is a rare congenital, multisystemic, hamartomatous condition characterized by asymmetrical overgrowth of almost any part of the body and a broad spectrum of manifestations. The developmental abnormalities associated with this syndrome can lead to unusual look and overgrowth of different parts of the body. The cause of the disorder is a mosaic variant in a gene called AKT1. Proteus syndrome is a rare disorder with a genetic background that can cause tissue overgrowth involving all three embryonic lineages. It is a complex disorder with multisystem involvement and great clinical variability. Patients with Proteus syndrome tend to have an increased risk of embryonic tumor development. The condition is characterized by the overgrowth of skin and bone development. This Greek god was capable of changing his shape.

Proteus syndrome is another form of disease that is rare and congenital in origin. Disproportionate, asymmetric overgrowth occurs in a mosaic pattern (i.e., a random "patchy" pattern of affected and unaffected areas). Proteus syndrome or Wiedemann’s syndrome is a congenital disorder resulting in overgrowth of body parts along with abnormalities of bone and skin. Proteus syndrome (PS) is a complex hamartomatous disorder consisting of disproportionate, asymmetric overgrowth of any tissue of the body, particularly the skeleton, cerebriform connective tissue nevi, epidermal nevi, vascular malformations, and dysregulated adipose tissue (Cohen, 2005; Biesecker, 2006).
It involves a congenital malformation disorder where an organ or tissue overgrows in a patchy distribution. The condition is also referred as Wiedemann’s syndrome, coined after Hans-Rudolf Wiedemann. Proteus syndrome is a rare disorder characterized by overgrowth of various tissues of the body. It is suspected to be a genetic condition, but a particular gene is not currently identified. Proteus syndrome is a rare condition that can be loosely categorized as a hamartomatous disorder. The growth is not symmetric, which means that the parallel parts of the body are not of the corresponding length of the other.


Proteus Syndrome was first described and categorized by Doctor Michael Cohen in 1979 and was named after the Greek god Proteus. There is also growth of the body tissues that makes the affected grow in size asymmetrically. Yet, the orthopedic manifestations of the syndrome are unique.
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