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Proteus syndrome

Background

Proteus syndrome is a rare disorder first identified in 1979 but with cases described in the literature since 1856.

The clinical features of Proteus syndrome are overgrowth/enlargement of soft tissue and bone which can affect any area of the body but often involving the hands and/or the feet, the skull and sometimes the whole of one side of the body (hemihypertrophy) and blood vessel (vascular) abnormalities. Superficial warty birthmarks (epidermal naevi), soft deeper (subcutaneous) lumps, thickening of the skin on the soles and bony problems, in particular, of the skull, hand/feet and curvature of the spine may also be present.

The abnormalities seen in Proteus syndrome are present at birth but may become more apparent and develop with age. The clinical features described may not all be present in any one affected individual and the severity can vary widely. The nature of the varied complications of the disorder depend on the site and severity of the problem.

How is it treated? View How is it treated?

Medical text written November 1998 by Dr J Harper. Last reviewed October 2004 by Professor J Harper, Consultant in Paediatric Dermatology, Great Ormond Street Hospital, London, UK.

 

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