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What is proteus syndrome?Proteus Syndrome is a congenital condition - the abnormalities are defined at birth, although some may not be fully apparent and will develop with age. Complications are variable and depend upon the nature of the problem, the site and the severity.Proteus Syndrome shows itself by an asymmetrical - one-sided - overgrowth of almost any part of the body together with a variety of other tissue abnormalities, including warty lesions on the skin, soft tissue lumps derived from blood vessel tissue, fatty lumps or a mixture of different types of tissue. The most characteristic forms of overgrowth are asymmetrical enlargements of the skull, the part or whole of one or more limbs and typically one or more large digits on the hands or feet. Another feature often seen is the ridged thickening of the soles (moccasin feet). Other aspects can include skeletal abnormalities such as bony lumps and spinal deformities. No two cases of Proteus Syndrome are ever identical, nor the severity, which can range from being very mild to severe, with the majority of cases falling into the mild to middle range. Medical care of a child with Proteus requires management across a number of medical specialists, including orthopaedic, genetic, ophthalmic, dermatolgical, general and plastic surgery, maxillary and facial, physiotherapy and psychology. Why is it called proteus syndrome?It was in 1983 that a German paediatrician, Rudolf Weidermann, gave the name of Proteus Syndrome to this recognised and unique collection of varying symptoms and abnormalities. The name was taken from Greek mythology, to emphasise the polymorphic nature of this condition - Proteus, the Old Man of the Sea, who could change shape at will to avoid capture.
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Secretary: Jean Harrison, 31 Baswich Lane, Stafford ST17 0BH 01785 254 953, jean@proteus-uk.org |
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