Treatment of Scleroderma

About the disease

Scleroderma is a disease, when skin and connective tissue hardens and tightens. It usually originates in fibers, responsible for providing the framework to the body of a human. Scleroderma is considered to be a very rare disease. Usually it affects women between 30-50 years of age. In 20% of cases scleroderma damages only the skin of a person. In other 70% structures beyond the skin, such as blood vessels, are also affected. In rare cases scleroderma damages internal organs of a person, most often digestive tract.

100% of all scleroderma cases develop because of abnormal production of collagen in the skin. As a result, collagen accumulated in the skin, causing separate tissues to tighten. Overall, collagen is a special type of protein, responsible for forming connective tissues in the body of a person. It is not known why collagen starts to be produced abnormally. It is believed that disruption in the immune system plays crucial role in collagen overproduction. Exposure to dangerous substances, such as pesticides or solvents, can also trigger development of scleroderma.

Symptoms

• Hardened patches on the skin in various areas
• Numbness in certain parts of the skin
• Skin becomes shiny in hardened places
• Color of the skin changes
• Pain in the affected areas
• Unusual response to cold temperatures

Diagnosis

It is difficult to diagnose scleroderma, as it can originate in different parts of the body.

• During physical exam doctor checks the skin to determine which areas exactly are hardened. He also determines response of a patient to cold temperatures.
• Blood test is necessary to be done to determine if there is increased response from antibodies which is an indicator of problems with immune system.
• X-Ray of the lungs and heart if done to see if scleroderma damaged these organs.
• Biopsy of the skin tissue of hardened part is done to check the skin cells under microscope and see if there are any problems.

Treatment

• Conservative treatment aims to control scleroderma and prevent development of any complications. It includes dilation of the blood vessels, which can prevent spread of the scleroderma to heart and kidneys.
• Suppression of the immune system for a certain period of time can decrease the production of collagen and thus eliminate scleroderma symptoms.
• Reduction of the stomach acid is performed if scleroderma affected digestive tract, thus causing the amount of stomach acid to increase.
• Lung transplantation is used in extremely rare cases if scleroderma affects the lungs to the extent, when a person needs transplantation.

Authors: Dr. Nadezhda Ivanisova, Dr. Farrukh Ahmed