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Scleroderma
What are the symptoms?
Scleroderma is divided into localised forms (morphoea, linear scleroderma) and generalised scleroderma (systemic sclerosis, SSc). SSc has a severe form with patients having wide spread skin involvement and a mild form with patients having skin involvement only in the hands, lower arms, feet, lower legs and face. The severe form, diffuse cutaneous SSc, affects forty per cent of sufferers; the mild form, limited cutaneous SSc, sixty per cent. In the severe form, wide areas of the skin are affected and internal organ involvement occurs early. Scleroderma can be fatal, with lung disease as the chief cause of death. Survivors may have a reduced quality of life with breathlessness, kidney disease, heart disease, digestive problems and reduced function in joints, muscles and hands. In the mild form, there is limited skin involvement with the later development of gut disease (and thus difficulty in swallowing and eating) and lung problems, usually pulmonary hypertension. Additionally there may be calcinosis (deposits of calcium which mass under the skin and protrude), dry eyes and mouth, ulceration and a very poor circulation.
Juvenile scleroderma differs from the adult disease in that localised forms predominate. The juvenile disease attacks particularly the skin, muscles, joints, tendons and bones, with internal organ involvement a rarity. Childhood morphoea may last three to four years then resolve spontaneously, but the linear form may lead to growth defects. The UK Scleroderma Childhood Register is based at the ARC Epidemiology Unit, University of Manchester. The register is funded by the Raynaud's & Scleroderma Association which supports children with Scleroderma and Raynaud's Phenomenon.
Background
| Inheritance patterns and prenatal diagnosis 
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