Proteus syndrome


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Pro·te·us syn·drome

[MIM*176920]
a sporadic disorder of possible genetic origin, having a variable and changing phenotype; characterized by gigantism of the hands and feet, by distorted, abnormal growth, pigmented nevi, thickening of the palms and soles, vascular malformations, and subcutaneous lipomas; often confused with neurofibromatosis type I.
Synonym(s): elephant man's disease (1)
[G. Proteus, a sea god who had the power to change his form]

Proteus syndrome

[prō′tē·us]
a rare congenital disorder with highly variable manifestations, including partial gigantism of the hands and feet with hypertrophy of the palms and soles, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly and other skull abnormalities, and abdominal or pelvic lipomatosis. The cause is unknown, although a genetic origin, possibly of autosomal-dominant transmission, has been conjectured. Although symptoms can be treated, there is no known cure.
A rare autosomal dominant [MIM 176920] condition characterised by acral gigantism, plantar hyperplasia, hemangiomas, lipomas, varicosities, linear verrucae, nevi, macrocephaly, cranial hyperostosis, pachydermy, and hypertrophy of long bones

Pro·te·us syn·drome

(prō'tē-ŭs sin'drōm)
A sporadic disorder of possible genetic origin, having a variable and changing phenotype characterized by gigantism of the hands and feet, distorted abnormal growth, pigmented nevi, thickening of the palms and soles, vascular malformations, and subcutaneous lipomas; often confused with neurofibromatosis type II.
Synonym(s): elephant man disease (1) .
[G. Proteus, a sea god who had the power to change his form]
References in periodicals archive ?
This disorder is characterized by such striking and varied deformities that Wiedemann and Associates called it Proteus Syndrome after Greek god Proteus (Meaning "polymorphous") who was capable of transforming into any shape to disguise himself and thus escape from his enemies.
The NIH Proteus syndrome team is headed by researchers who originally discovered the somatic single (point) mutation in the AKT 1 oncogene that causes Proteus syndrome.
At the time, she had not yet been diagnosed with Proteus syndrome.
Proteus syndrome presenting with hemihypertrophy consisting of lipomatous tumor is similar to macrodystrophia lipomatosa.
It is now believed to be a part of generalised hamartomatous disorder featuring fat in the soft tissues and even in the bowel, known as Proteus syndrome.
Isolated plantar collagenoma not associated with proteus syndrome.
Tonight's opener profiles Mandy Sellars, who was born with an extreme form of Proteus syndrome, which has left the 33-year-old with legs four times the normal size.
Tonight's opener profiles Mandy Sellars, above, who was born with an extreme form of Proteus syndrome, which has left the 33-year-old with legs four times the normal size.
The specialist operation at Cwmbach includes making shoes for Mandy Sellars, who suffers from Proteus Syndrome, an over-growth of the limbs, and other disabled people with unique needs.
He is severely retarded and has been diagnosed with Proteus syndrome, also known as Elephant Man syndrome.
Mandy has Proteus Syndrome, an incurable affliction which bloats limbs and joints to gigantic proportions and affects just 200 people worldwide.