Scleroderma is an autoimmune disorder; it is characterized by thickening of the skin and connective tissues. Scleroderma also affects the internal organs of the body. The body’s immune system attacks the skin, connective tissue and organs which causes the tissues to become thick and fibrous. Scleroderma can involve just certain areas of the body or it can be systemic and affect the entire body.
What causes scleroderma?
The exact cause of scleroderma remains a mystery, but researchers do know that the body’s immune system attacks the tissues of the body which causes scar tissue to form. It is believed that genes play a role; scleroderma seems to run in families. It’s fairly common for different types of autoimmune diseases to run in families where there is scleroderma. Scarring occurs when the connective tissues produce too much collagen. It is believed that stress can cause the manifestations of scleroderma worse.
What are the characteristics of scleroderma?
The characteristics of scleroderma may differ according to the degree of skin and connective tissue involvement. Scleroderma presents in different forms. The localized form of scleroderma is called limited scleroderma and the systemic form is called diffuse scleroderma. Limited scleroderma tends to be limited to the skin of the face and fingers only; it is slow to spread. Diffuse scleroderma affects the skin and connective tissues of the entire body; the face, trunk and extremities are affected. Diffuse scleroderma also affects the esophagus, heart, kidneys, lungs and bowels. People who have diffuse scleroderma may also have problems with maintaining a normal blood pressure.
Further characteristics of scleroderma include calcinosis, Raynaud’s phenomenon, esophagus disease, sclerodactyly, and telangiectasias, which will be discussed below.
Calcinosis – Calcinosis refers t hardening of the skin through calcium deposits forming on the skin. Calcinosis occurs on the bending joints of the body, such as the fingers, knees, and elbows. These areas can break open and become infected. Pieces of the hard skin can break off or partially break off at times. Sometimes pieces of calcified skin need to be surgically removed. In places where calcinosis has occurred can be quite tender and painful.
Raynaud’s phenomenon – Raynaud’s phenomenon is an uncomfortable part of scleroderma. With Raynaud’s phenomenon the tiny arteries which supply the toes, fingers, ears, tongue and nose go into frequent spasm and relaxation. When the arteries go into spasm, blood and oxygen are not allowed to enter those areas; as a result the areas without blood supply become pale and experience pain and cold. When the arterial spasm starts and stops, blood flow stops and resumes and the areas affected turn from white to blue to red. Raynaud’s phenomenon usually affects women more than men.
Esophagus disease – Esophagus disease affects the muscles of the lower section of the esophagus. If you were to divide the esophagus into thirds, the first third of the esophagus would function fairly normally, but the bottom two thirds would become wider from scarring and lack of muscle tone. Acid reflux is a common companion with people who have sclerodermal involvement in the esophagus. Acid reflux must be treated aggressively to prevent scarring of the esophagus.
Sclerodactyly – Sclerodactyly refers to the skin changes that take place with scleroderma. The skin becomes thick and shiny as it calcifies around the bendable joints. Sclerodactyly progresses faster in people with limited scleroderma than with diffuse scleroderma. The thickening of the skin can interfere with being able to move your joints, fingers and toes.
Telangiectasias – Telangiectasias is characterized by reddening of the skin. The areas may be tiny or larger in size. These reddened areas are caused by dilated capillaries in the skin. The areas may be commonly seen on the face, inside the lips, and on the palmer surface of the hands.
Scleroderma is chronic and degenerative during one’s life. Though, scleroderma can be uncomfortable, most people can lead fairly normal life spans. People with scleroderma may experience varying degrees of disability. People with systemic (diffuse) scleroderma have more likelihood of having kidney and heart involvement which can cause severe high blood pressure. If scleroderma affects the lungs, an individual may be more susceptible to breathing problems due to thickening of the tissues of the lungs. Thus, if scleroderma affects the digestive system, the individual may have problems with swallowing difficulties, weight loss from malabsorption of food in the small intestine, constipation and there may also be problems with fecal impactions or intestinal obstructions.
The symptoms and treatment options for scleroderma will be discussed in a future article.