Scleroderma, or systemic sclerosis (SSc), is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.
One of the hallmarks is the thickening or hardening of the skin. This is literally what the word means in Greek (sclero=hard, derma=skin).
However, the term systemic sclerosis indicates that:
- it is a systemic disease, that is, it may affect many parts of the body, and
- sclerosis is the medical term for the thickening or hardening of the tissues, which is a major part of this condition.
Scleroderma can cause serious damage to internal organs including the lungs, heart, kidneys, oesophagus, and gastrointestinal tract. As such, it is often referred to as a “multi-system” disease. It can be life-threatening. Scleroderma is three to four times more prevalent in women.
Scleroderma is not contagious, it is not infectious, and it is not cancerous or malignant.
The different types of Scleroderma
There have been many attempts to classify scleroderma so that patients and doctors can plan appropriate management of the condition.
This is because the condition varies widely in different patients, with some patients having only a very minor problem with never any progression of the disorder, whilst others can have a very serious illness. Luckily, the majority of patients have the milder disease.
Basically there are two types of scleroderma. The first is termed limited and the second diffuse. The extent of skin involvement is used to divide the patients into these two groups.
Limited scleroderma usually causes Raynaud’s phenomena and hardening of the skin in the hands. Other internal organ involvement can occur, though usually over a period of many years with the disease.
Diffuse scleroderma affects the skin not only on the hands and forearms, but it can also affect the skin on the trunk, upper arms and thighs.
Patients with this condition often have a more systemic illness affecting other organs and tissues and can require more intensive treatment.
About 70% of people with systemic scleroderma have limited disease, while approximately 30% have the more diffuse form.
Although most patients can be classified as having either limited or diffuse disease, different people may have different symptoms and different combination of symptoms of the illness.
What Causes Scleroderma?
The cause of Scleroderma is unknown. What we do know is that people with Scleroderma have activation of their immune system. In addition they are producing too much collagen and thirdly some of their small blood vessels become narrowed. It is the combination of these three events that produces the various clinical features of Scleroderma.
One suggestion about what causes scleroderma is that something in their body damages some of the small blood vessels. This in turn activates the immune system and the activated immune system makes chemicals that switch on the fibroblasts. These activated fibroblasts then produce excessive collagen.
Who suffers from Scleroderma?
Scleroderma affects both sexes, with a female to male ratio of between three and four to one. It can occur at any age although the peak incidence is 40-60 years. The condition can occur in children although this is rare. It has been reported in most countries throughout the world and in most racial groups. It is less common in Black Africa and Polynesia.
The condition is not hereditary and it is very rare for it to occur in more than one member of a family. There are estimated to be over 5,000 persons with scleroderma in Australia.