Scleroderma
Scleroderma is the persistent fibrosing and contraction of the body's connective tissue. It is also known as Progressive Systemic Sclerosis or PSS. The cause is unknown, but it is characterized by diffuse fibrosis, coupled with vascular abnormalities and degenerative changes in the skin, articular structures and some internal organs.
Onset can be insidious, but commonly the skin is the first area where noted changes have occurred. Raynaud's phenomenon is one clear example where the blood vessels in the hands or feet are abnormally sensitive to the cold, and blood flow is restricted causing co lour changes and numbness.
In Scleroderma, the skin changes and becomes taut, shiny and sometimes hyper pigmented. There is an imbalance in the tissue cell types that make up the skin structure, with an increase and thickening of collagen fibres (the fibrous tissue), and a thinning of the epidermal layer (the outer skin layer). Subcutaneous calcification can occur, particularly on the fingertips and elbows.
The abnormal contraction element of this condition can cause hyper flexion distortion at the wrists, fingers and elbows.
When the organs are affected it causes excessive fibrosis in tissues that rely on their ability to expand easily e.g. lungs and heart. Fibrosis causes stiffening in the tissue, in the lungs this will lead to a loss of expandable area for gas exchange and Pleurisy is common due to the poor movement of the lungs and the sluggish blood supply.
The kidneys if affected can be irreversibly damaged and usually extremely high blood pressure which cannot be controlled safely is one of the common symptoms.
Scleroderma is often seen in patient's suffering from Rheumatoid Arthritis and a condition known as Systemic Lupus Erythema or SLE. There is no cure that can reverse or stop the problem, however it is possible to locally treat some symptoms with steroids or specific drugs aimed at slowing down the various changes.
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