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 ?
At the time, she had not yet been diagnosed with Proteus syndrome.
The diagnosis could be suspected on plain radiograph but MRI is the better diagnostic imaging modality as it can rule out other differential diagnoses such as neurofibromatosis, haemangiomatosis, lymphangiomatosis, Proteus syndrome and fibrolipomatous hamartoma.
Merrick suffered from major skin and bone abnormalities, believed to have been caused by Proteus Syndrome.
The seven-year-old pupil of Corpus Christi RC Primary School, Ernesford Grange, has grown up knowing his mum Paula suffers from a rare medical condition called Proteus Syndrome.
Based on published clinical features, cancer geneticists and other clinical geneticists believe that Merrick had at least Proteus Syndrome.
But in 1987, the National Institutes of Health concluded that Merrick instead had the extremely rare Proteus syndrome.
Proteus syndrome may be similar but has other associated features such as skull anomalies, lung cysts and pigmented naevi.
Medical curiosity who became disfigured by what is now known as Proteus Syndrome, and was otherwise known as The Elephant Man.
Some medics think she has a rare disorder called Proteus Syndrome, a condition that causes skin overgrowth and atypical bone development, often accompanied by tumours over half the body.
She is thought to suffer from Proteus Syndrome, a rare disease most commonly associated with Joseph Merrick, alias the Elephant man.
Dillon, who is four years old, suffers from a rare disease called Proteus syndrome and the money raised will be used to send him to see specialists in the United States.