Approximately 300,000 people in the U.S. have scleroderma. Women are affected three times as often as men. Although the disease can develop at any age, it most frequently appears in the third to fifth decades of life. It is most frequently a chronic and often progressive illness.
Collagen, a protein manufactured by the connective tissues of the body, is produced in excess in someone suffering with scleroderma. This over-production of collagen can be likened to the process of "scarring," which is the way the body heals a wound.
For persons with scleroderma, the production of collagen is abnormal, depositing it in various organs and/or tissues of the body, especially in layers of the skin.
It is important to stress that the symptoms of scleroderma vary greatly from person to person, as though each person with scleroderma has his/her own version of the disease. Although scleroderma can indeed be serious, most people are able to live a normal life span with varying degrees of discomfort and/or disability.
There are two forms of scleroderma: localized and generalized (also called systemic sclerosis).
- affects mainly the skin in different areas of the body
- may affect muscles and bone
- does not affect internal organs
- is not usually as severe as generalized scleroderma
- does not usually develop into generalized scleroderma
Generalized scleroderma affects the skin and/or internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints.
Generalized scleroderma most often presents as either CREST syndrome or diffuse scleroderma.
CREST is an acronym derived from the syndrome's five most prominent symptoms:
- C - calcinosis, painful calcium deposits in the skin
- R - raynaud's phenomenon, abnormal blood flow in response to cold or stress, often in the fingers
- E - esophageal dysfunction, difficulty swallowing caused by internal scarring
- S - sclerodactyly, tightening of the skin on the fingers and toes
- T - telangiectasia, red spots on the hands, palms, forearms, face and lips
Diffuse scleroderma appears as thickening of the skin that often spreads from the fingers and hands to the face, trunk or major organs.
Other less common forms of scleroderma include chemically induced localized scleroderma, eosinophilic myalgia syndrome (a disorder caused by the ingestion of L-tryptophan), toxic oil syndrome (associated with contaminated oil), and graft-versus-host disease.
Effects Of Scleroderma On The Body
- Scleroderma in the skin may cause the cessation of hair growth and sweating; and the tightening and thinning of skin over the knuckles or finger joints, resulting in ulcers (open sores) and curving of the digits.
- Scleroderma in the kidney may cause severe hypertension (high blood pressure) and kidney failure.
- Scleroderma in the gastrointestinal tract hampers the action of the smooth muscle that lines the esophagus, small intestine, and colon; resulting in swallowing difficulties, malabsorption of digested food into the circulation, severe constipation and/or possible intestinal obstruction.
- Scleroderma in the lung makes the lungs less flexible and reduces their blood supply resulting in shortness of breath, susceptibility to bronchial problems, and pulmonary fibrosis (scarring and thickening of lung tissue).
- Scleroderma in the heart and pericardium (fibrous sac that lines the heart) restricts the normal pulsation and pumping of the blood by encasing the heart in a shell of sclerosed tissue. This may lead to heart failure.
- Scleroderma in the joints causes arthritic symptoms such as inflammation, pain, swelling, stiffness, redness, and, in some cases, joint deformity.