What is scleroderma?
Scleroderma is a medical condition characterized by thickening of the connective tissue of the body that provides supporting frame work to the body. There are two forms of scleroderma, one is localized involving only the skin and another form is systemic affecting different internal organs of the body like heart, lung and kidneys.
This condition does not spread from person to person although in some cases there may be prior family history of scleroderma. Outcome of the disease is generally good in patients suffering from localized form whereas it is not so good in elderly patients suffering from the diffuse form of the disease.
Symptoms of scleroderma depend upon the organ involved. There are two principal forms of scleroderma, localized and diffuse forms.
Localized and Diffuse
Localized form is characterized by :
- Appearance of characteristic skin lesion known as morphea with a white central area and purple border. Usually these skin patches may clear within 3 to 5 years the affected area may appear darker in comparison to adjacent normal area.
- Linear scleroderma is appearance of band like thickening of the skin especially in the arms, legs or forehead region especially on one side of the body. Usually children suffering from scleroderma show this type of skin lesions.
Symptoms of localized scleroderma may occur along with other associated diseases like :
- Raynaud’s phenomenon characterized by pain, numbness and color change of the tips of finger due to constriction of smaller blood vessels in skin after exposure to cold.
- Gastroesophageal reflux disease characterized by heart burn as acid from the stomach enters the esophagus due to impaired functioning of the valve of the lower part of the esophagus.
- Skin appears shiny and tight around hand, face and mouth region restricting movement of those area.
Symptoms of diffuse scleroderma include involvement of other internal organs besides skin leading to impaired function of these affected organs.
Complications of scleroderma may lead to ulceration in the fingers in Raynaud’s disease, scarring of lung tissue leading to respiratory distress, impaired kidney function ultimately leading to kidney failure, impaired heart function leading to rhythm abnormality even heart failure, as the skin surrounding the oral cavity becomes tight difficulty in maintaining adequate dental hygiene leads to number dental problems, sexual dysfunction like erectile dysfunction in men and inadequate lubrication during sexual activities lead to pain during intercourse in women.
Causes of Scleroderma
The exact cause of scleroderma is not known but for some reason the immune system of the body starts acting against own body cells regarding them as foreign cells and produces excessive amount of collagen. Collagen is the tissue that is responsible for maintenance of elasticity of skin and other organs, excessive collagen accumulation leads to tightening and thickening of the skin and symptom of scleroderma.however
There is no permanent cure for scleroderma. Common treatment options include drugs to dilate blood vessels to relieve symptoms of kidney and lung involvement, suppressing the immune system, surgical procedures like amputation, transplant of lung and physical and occupational therapy.