A chronic autoimmune disease that mainly affects the skin and can also affect blood vessels, internal organs, and joints. Scleroderma is characterized by excess collagen, which is the main supporting protein in connective tissue. The origin of the term “scleroderma” in Greek means “hard skin” (sclerosis = stiffness, drama = skin). This term indicates hardness and thickening of the skin, which is a clear sign of illness.
Scleroderma has two main forms:
Limited Scleroderma or Morphea Morphea – Limits cutaneous scleroderma A milder condition that mainly affects the hands, arms, and face, and sometimes also includes pulmonary hypertension.
The second and more severe form is scleroderma, or systemic sclerosis, a more rare disease that develops rapidly and affects large areas of the skin, as well as internal organs such as the kidneys, esophagus, heart and lungs, and can be fatal.
There is no specific treatment for scleroderma, but there is treatment for the complications it causes. The cause of scleroderma is unknown. It exists in families (a familial disorder), but the genes involved have not been fully identified. The disease affects small blood vessels – arterioles – in all organs. At the onset of the disease, the endothelial cells lining the arteriole die along with the smooth muscle cells that are part of the blood vessels during apoptosis. These cells are replaced by collagen tissue and fibrosis. Inflammatory cells, especially CD4 + helper T cells, invade the arteriole, causing further damage. Medicines used for the disease block the inflammatory processes that occur through this mechanism.
