Monthly Issue--- Scleroderma

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Scleroderma

What is Scleroderma ?--
Scleroderma literally means "Hard skin". As the name suggests it is characterized by abnormal thickening of the skin. The same thickening can occur in the internal organs.

What are the different types of Scleroderma ?--
Diffuse Scleroderma --- Skin of multiple regions is thickened. This type of Scleroderma is associated with early involvement of the internal organs.
Limited Scleroderma (CREST syndrome)-- This form has a slower progression.
Linear Scleroderma & Morphea -- these forms are localized to the skin & internal organs are not involved.

What causes Scleroderma ?--

Scleroderma is thought to be caused due to disordered immune system. The immunity of a person is programmed to act against foreign agents. However in the case of Scleroderma, it fails to recognize the body's own organs & mounts attacks against them considering them to be foreign agents. Antibodies to microscopic components of the cell are formed. These include anti centromere, anti topoisomerse I etc.

What are the skin changes associated with Scleroderma ?--

Scleroderma is associated with progressive tightening of the skin. The tightening then causes decreased flexibility especially at the joints. These changes are particulary seen on the hands, face especially around the mouth.
Scleroderma is also associated with Reynaud's Phenomenon. For details click here.

What are the symptoms of Scleroderma ?--

The other symptoms depend on the system involved -------

Respiratory system -- this is characterized by progressive shortness of breath after exercise or other exertion. Early involvement can be detected by pulmonary function tests as X ray may be normal during the early cases.
Gastrointestinal system -- Skin thickening around the mouth can lead to difficulty in opening the mouth. Further involvement of the esophagus leads to difficulty in swallowing & heartburn. In this case the early involvement of the esophagus can be detected with manometry of the esophagus.
Heart --- Generally manifests as irregular rhythm, missed beats etc. However heart involvement is much less common. Many patients also have a high blood pressure.
Kidney-- Generally does not produce symptoms for a long time.
Other -- joint pain, muscle stiffness does occur.

What is the treatment of Scleroderma ? ---
As Scleroderma is caused by immunological disturbances, hence the treatment involves use of immunomodulator drugs. These include cyclophosphamide, azathioprine. Steroids are used only in specific circumstances & not as a routine for every patient.