Scleroderma is an uncommon condition that affects the skin and connective tissue in such a way that they tighten up and contract down. It is a progressive disease that worsens over time. It often starts out as a dry patch of skin that becomes thickened. More patches tend to appear with time and spread. “Sclero” means ‘hard,’ and the “derma” portion refers to the skin.

Scleroderma is an “auto-immune” condition where the thinking is that the body makes antibodies against itself, and in this case it’s the connective tissue the antibodies target. The inflammatory response of the skin creates an over-production of collagen and thus creates the tightening. Scleroderma can affect every organ, but the skin is the most common area to cause symptoms. Most of the time scleroderma happens in a person that has no family history of the disease, but it can run in families as well.

As mentioned earlier, the first sign of scleroderma is normally a thickening and hardening of the skin in a patch which spreads and increases in size. The skin around the mouth can start to look really tight, and in other places it loses its elasticity. This can make the skin appear shiny. With the digestive tract, people with scleroderma can tend to have problems with digestion as the food is not moved along properly and therefore not absorbed properly. Joint pain often comes, and there can be puffiness of the joints, especially in the morning. The fingers can turn white and numb with cold or stress (“Raynaud’s Phenomenon”).

There are a few different types of scleroderma and they can be divided into “localized” and “systemic.” The localized versions are: Morphea and Linear Scleroderma. With Morphea a person develops purple ovals that are thick around the edges with a central whitish clearing. They tend to be on the trunk, but can appear elsewhere. With Linear Scleroderma there are bands or lines of thickened skin on the limbs and sometimes the forehead. The systemic versions of Scleroderma include: diffuse cutaneous systemic sclerosis, limited cutaneous systemic sclerosis, and sine scleroderma. The diffuse variant is the most serious, affecting many different organs including the lungs, heart, kidneys, and intestines. If left untreated this variant can be fatal. With the limited cutaneous type the disease affects the skin of the lower arms, lower legs, face, and neck. In sine scleroderma the disease affects other parts of the body not including the skin.

Scleroderma can be a difficult diagnosis to make. Your doctor will review your history and do a physical examination. A tissue biopsy from your skin may be taken to be examined under the microscope and blood tests may be done to look at antibody levels.

Treatment for scleroderma is mainly to reduce any inflammation. There is no cure. Anti-inflammatories are useful and immuno-modulatory medications (reduce the immune response) can help. As you can imagine though, there are side effects with the latter.

People with scleroderma need to be closely monitored by physicians who specialize in the skin and in joint problems, among others. Your family doctor is key in coordinating the specialist treatment that is necessary.