Scleroderma is a chronic autoimmune disease characterized by patches of scar-like thick skin. This rare connective tissue disorder is caused by the buildup of a protein called collagen, the main component of scar tissue.
Scleroderma, also known as systemic sclerosis, appears to develop spontaneously, and its underlying causes are largely unknown.
Collagen is the most abundant protein in the body. A structural protein, collagen is deposited between cells to help keep tissues firm and flexible. It is produced by specialized cells called fibroblasts, which can also recognize and produce chemicals that allow them to communicate with the cells of the immune system.
After an injury, both the immune system and fibroblasts are activated as part of the wound healing process. The immune system protects the injured tissue by keeping disease-causing viruses and bacteria away, while fibroblasts secrete collagen, creating a scab to rapidly close the wound. Once the danger has passed and damage is repaired, the milieu of chemical signals shifts, which normally stops the inflammatory immune reaction and the excessive collagen production by the fibroblasts.
In people with scleroderma, the body keeps producing collagen as if there was a wound that constantly needs to be repaired. Scleroderma most often affects skin, but other organs can also be damaged as a result of collagen accumulating in tissues.
Scleroderma and genetics
Scleroderma does not have a known genetic cause, and it is not classed as a hereditary disease — meaning that a person cannot pass the disease to their biological children. Still, those with scleroderma often have a family member with another autoimmune disease, like lupus or rheumatoid arthritis, implying that autoimmune diseases may have a heritable element.
More biological females — about four times as many — are affected by scleroderma than males, and the disease most commonly develops between ages 35 and 50. This distinction in age and sex may indicate an unidentified connection between the disease and a person’s hormonal state.
In the U.S., Black and Native Americans are more often affected by scleroderma than whites. While socioeconomic factors likely play a role, these racial differences are also thought to reflect differences in genetic susceptibility to scleroderma between ethnic groups.
Some evidence points to possible environmental triggers of scleroderma. For example, infections by some viruses and long-term exposure to some chemicals, such as pesticides, silica dust, or polyvinyl chloride, are thought to be linked to the disease.
Variations in the type of exposure and disease symptoms, however, make it difficult to establish a clear cause-and-effect relationship between such triggers and scleroderma.
Last updated: May 12, 2021
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