Doctor's Notes on Systemic Scleroderma
Systemic scleroderma (also called systemic sclerosis) is a chronic autoimmune disorder that affects the connective tissues of the skin and internal organs. Systemic scleroderma is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The scarring is due to the excess production of the protein collagen, which normally strengthens and supports connective tissues throughout the body.
Early symptoms of systemic scleroderma include episodes of Raynaud phenomenon, in which the fingers and toes turn white or blue in response to cold temperature or other stresses; and puffy or swollen hands before the skin thickens and hardens due to fibrosis. Skin thickening usually occurs first in the fingers and may also involve the hands and face. Other symptoms of systemic scleroderma include open sores on the fingers, painful bumps under the skin, or small clusters of enlarged blood vessels just under the skin. Fibrosis can also affect internal organs and can lead to damage or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys. Symptoms of internal organ fibrosis due to systemic scleroderma may include heartburn, difficulty swallowing, high blood pressure, kidney problems, shortness of breath, diarrhea, or impairment of the muscle contractions that move food through the digestive tract.
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Raynaud SyndromeRaynaud's syndrome (also called Raynaud's phenomenon) is a blood vessel disorder. The smooth muscle in the walls of arteries contract, causing "vasospasms" of the small vessels that supply blood to the extremities. The typical features of include changes in the color of skin from pale to bluish to red, which typically occur after exposure to cold temperatures. The disorder is sometimes referred to as "white fingers disease."
Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.