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Scleroderma is a disease that typically involves abnormalities of the skin. It is also a systemic disease, meaning that it has the potential to involve internal organs of the body.
The features of the illness vary greatly from patient to patient. Nevertheless, it has been found that scleroderma can manifest according to certain patterns. These patterns have important differences in the frequency and distribution of involvement of various organs, as well as prognosis.
By far the most common patterns of scleroderma are those referred to as diffuse and limited. Patients with the diffuse form can have involvement of skin with thickening on the trunk of the body as well as the face and extremities. Substantial internal organ involvement is more frequent and can be severe. Those with the limited form have skin thickening that is "limited" to the extremities beyond the elbow and knees (fingers and feet often affected) face and neck. Internally disease is usually limited to the esophagus, although some develop a late lung disease.
A third pattern of scleroderma has been described, scleroderma sine scleroderma. This form of scleroderma is unique in that patients can have any of the characteristic features of internal organ involvement WITHOUT having detectable skin features. Scleroderma sine scleroderma is extremely rare. Patients can have any of the reported features of disease of the bowels, esophagus, lungs, heart, and/or kidneys. In an individual patient, treatment is customized according to the extent and severity of involvement of these internal organs.
It is important to emphasize that scleroderma can present with overlap features of any of the above patterns in a given individual. It does not always differentiate into specific patterns, although it usually does.