Scleroderma - CREST syndrome - Signs - Symptoms - Diagnosis - Treatment - Prognosis

Other Names:- CREST syndrome; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma

[Greek:- Sclerosis’ meaning ‘hardness’ and ‘derma’ meaning ‘skin’]

'Scleroderma' refers to a chronic, auto immune disease, although in reality, the word indicates just a single symptom (dry skin) of the grave condition.

Scleroderma can affect anyone, but it has been generally observed that women acquire it more than men. Children too can develop the disease.

Scleroderma is classified both as a rheumatic and a connective tissue disease. It is a connective tissue disease because in some types the skin becomes hard and fibrous, while in other types, the disease is much more intense as it affects the connective tissues of the internal organs such as heart, lungs or the kidneys. In the latter, the disease is often fatal.

Scleroderma also causes inflammation in the joints and muscles which is why it is a rheumatic disease as well.

Individuals who are affected by scleroderma may have the localized form or the systemic form of the disease. In localized scleroderma, the disease affects the skin of the face and hands, while in the systemic form the disease spreads to the internal organs too.

The localized scleroderma manifests before the age of 40 years and is more common among those with a European lineage than among other ethnic groups. The systemic type, meanwhile, manifests between the ages of 30-50 years and is more common among the African Americans.

It is estimated that approx. 250 people per million have some form of scleroderma.

The causes of scleroderma are not clear. It is known to run in families but the genes concerned have not been, thus far, identified. It can affect the quality of life of a person, greatly affect his self-esteem and the ability to accomplish everyday tasks.

The disease first affects the arterioles or the small blood vessels of body organs. The endothelium (inner lining) of the arteriole along with the smooth-muscle cells is the first to deteriorate and die due to a process known as apoptosis. During the process, cellular material is replaced by fibrous tissues, such as collagen. This is followed by the entry of CD4+ helper T cells, and other such inflammatory cells, into the arteriole, resulting in further damage.

The symptoms of scleroderma often overlap with those of other autoimmune diseases; therefore it is important to rule out other diseases.

Research has helped in identifying the vast majority of inflammatory and destructive protein signals that are associated with the disease; these signals have been regarded as potential targets for developing drugs against the disease. Treatment for scleroderma is largely  focused on controlling its symptoms.

Limited scleroderma has a better prognosis in comparison to the diffuse type. Once the disease spreads to the internal organs, prognosis is rather poor.