SCLERODERMA
Scleroderma (with a hard C) means tough skin. Fibrous tissue is deposited in the connective tissue that underlies the skin and the covering and lining of other organs - blood vessels, lungs, heart, kidneys etc.
The cause is not known. “Scleroderma like” conditions have been noted in people exposed to silica (in mining), and some industrial solvents. Many patients recall an extended period of emotional stress prior to diagnosis. The implication being that this stress has been the ‘trigger’ that caused annoying ‘ill-health’ to develop into symptoms which made a clinical diagnosis possible.
Diagnosis is difficult, certainly in the early stages. Diagnosis is based on clinical observation and laboratory tests. There is no specific set of symptoms or laboratory test that can point to the disease. Raynaud’s phenomenon (q.v.) is present in 99% of people affected.
The spectrum of disorders encompassed in the term scleroderma include:
* Morphea, also known as localised or linear scleroderma. This is usually limited to a specific area of thickened skin, although internal organs can be affected.
* Juvenile scleroderma which is usually localised, but may develop to systemic.
* Systemic sclerosis with two major subsets:
* Limited cutaneous systemic sclerosis,
* Diffuse cutaneous systemic sclerosis.
Accepted statistics for systemic sclerosis show a prevalence of 1 in 50 000 of population. Recent and ongoing research indicates that this may be understated by a factor of 10. That is, a prevalence of 1 in 5 000 is probable. This does not imply an increase in the disease, rather an increase in awareness and in the diagnosis of cases. Most people diagnosed will be over 40 years old. The female/male ratio is 4:1.
Systemic sclerosis is a life threatening disease for which there is no cure. Treatment is limited to the easing of symptoms and slowing progression. Sudden kidney failure has been the worst problem in the past. Dialysis and kidney transplantation have been successful in reducing this from being the major cause of death. Lung involvement is now considered to be the worst condition that may develop.
Anyone with Scleroderma should be seeing a rheumatologist or immunologist. Apart from the GP, who should coordinate treatment, patients might attend a gastroenterologist, ophthalmologist and dermatologist. Other specialists may also be involved.
