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Scleroderma is a connective tissue disorder that is characterized by hardening and induration of skin. Scleroderma itself is a manifestation of systemic sclerosis, an autoimmune disease involving skin, subcutaneous tissue, muscles, and bones. There is excessive deposition of connective tissue macromolecules in the skin that causes thickening. It is not an inherited disease, but genetic factors play an essential role in disease onset and progression.
Systemic sclerosis is divided into the following types depending upon the extent of the disease:
Systemic sclerosis is often misdiagnosed, so the exact number of cases is not well known. It is more common in black people. It is 4 to 9 times more likely to occur in females than in males for unknown reasons. The most common occurrence is in the age group 30 to 50 years.
Following are the signs and symptoms of scleroderma:
Complications of scleroderma include:
Scleroderma is usually diagnosed based on clinical symptoms. Some laboratory investigations that aid in the diagnosis are:
Following clinical conditions should be considered in differential diagnoses of scleroderma:
The mainstay of treatment is improving the quality of life by managing symptoms and complications.
The prognosis for limited and diffuse cutaneous types is good. The factors associated with poor prognosis of scleroderma include:
Scleroderma is a connective tissue disorder that is characterized by hardening and induration of skin. Scleroderma itself is a manifestation of systemic sclerosis, an autoimmune disease involving skin, subcutaneous tissue, muscles, and bones. There is excessive deposition of connective tissue macromolecules in the skin that causes thickening. It is not an inherited disease, but genetic factors play an essential role in disease onset and progression.