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| What is Scleroderma? |
Scleroderma is a chronic connective tissue disease and is generally classified as one of the auto-immune rheumatic diseases.
To grasp some idea of this disease it is helpful to understand the origin of the word, ‘Scleroderma’ is Greek - "sclero" meaning thick and "derma" meaning skin. It is where the body’s immune system does not adequately protect the connective tissues – that is, the tissues that hold the muscles, blood vessels, fat and skin together.
The pattern of the disease is also unusual in that it affects people in many different ways and the range and extent of symptoms may vary from one person to another.
Who is affected by Scleroderma?
Scleroderma affects both sexes, with a female to male ratio of around three to one. It can occur at any age although the peak incidence is 40-60 years. The condition can also 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.
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 excess collagen.
What are the different types of Scleroderma?
There are two main types of Scleroderma. The first main type is termed limited and the second type is termed diffuse. The extent of skin involvement is used to divide the person with Scleroderma into these two main groups. In general, the skin involvement in Scleroderma begins at the fingers and spreads up the arms. Some thickening of the skin of the face is very common, and in some people the legs are also involved. People are classified as limited if, in addition to the involvement of the face, there is thickening of the skin from the hands only to the elbows, and in the legs, if the thickening extends from the foot only as far as the knee. Individuals with Scleroderma are classified as diffuse if there is more extensive spread of the skin thickening, that is, the skin of the upper arms, thighs or trunk is involved.
Limited Scleroderma usually causes Raynaud’s Phenomena and hardening of the skin in the hands. There may be some changes in the facial skin and as indicated above, occasionally there is thickening of the skin on the forearm and lower leg. Oesophageal problems are common. Occasionally there is other internal organ involvement, but it is important to realise that this involvement is often very mild and may occur only after many years of the disease. The onset of limited Scleroderma is often slow and any progression of skin involvement is also very slow occurring after many years. The outlook for limited Scleroderma is very good.
Diffuse Scleroderma not affects the skin only on the hands and forearms but it can also affect the skin on the trunk, upper arms and thighs. People with this condition often have a more systemic illness with the Scleroderma process potentially affecting many other organs and tissues. This type of Scleroderma often requires more intensive treatment, and some people with this type have a serious disorder. Diffuse Scleroderma generally has a fairly rapid onset of the disease with the skin thickening spreading rapidly over a few months. However, skin thickening can remit after several years with little long-term damage, assuming the patient did not have any significant problems in the first few years of the disorder when it was active.
Overlap syndrome occasionally may occur when Scleroderma is present in conjunction with another connective tissue disease (e.g. Systemic Lupus Erythematous or Polymyositis).
CREST is another name sometimes used to describe a sub-group of people with Scleroderma. This term was more commonly used in the past but nowadays it is used less commonly as classification into limited and diffuse has been found more useful in predicting long term outlook. CREST is the acronym for the clinical combination of Calcinosis, Raynaud's Phenomena, Oesophageal problems, Sclerodactyly (stiff fingers) and Telangesctasia (small dilated red vessels in the skin of the hands or face). Most people with CREST have limited Scleroderma.
Morphea is a condition related to Scleroderma that is sometimes called localised Scleroderma. In this condition there are localised patches of thickened skin. Those with this condition do not have Raynaud’s and do not have any internal organ involvement.
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