Systemic Sclerosis (SSc) or systemic scleroderma is a rare disease characterized by hardening of the skin due to a build-up of fibrous scar tissue, causing a tight and shiny appearance. The most common area affected is the fingers but it can happen in many other areas. In some cases other connective tissues or organs like the lungs and kidneys are affected, leading to a wider range of symptoms.
There are two forms of systemic sclerosis: limited and diffuse. In the limited form, skin involvement is limited to lower parts of the arms and legs, and the face and neck. In the diffuse form, skin involvement can be far more widespread.
Systemic sclerosis is an autoimmune connective tissue disease, meaning it is caused by the body’s immune system attacking its own skin and connective tissues. The reason why it does this is not well understood. When the body’s immune system is “activated” in this way, it can make a person feel very tired, similar to when they have the flu.
Almost all people with systemic sclerosis have a condition called Raynaud’s phenomenon. This causes the fingers and toes or the tips to turn white and then blue and/or red in the cold because of an over-reaction to cold temperatures.
SSc is very rare. It affects about 1 in every 100,000 people. About 90% of cases are seen in women who are often aged between 40 and 60 years, but it can start at any age. It is extremely rare in children. It can be difficult to diagnose the disease when it is in its early stages.