Scleroderma (cont.)

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What are scleroderma symptoms and signs?

The symptoms of scleroderma depend on the type of scleroderma present and the extent of external and internal involvement in the individual affected. Because scleroderma can involve the skin, esophagus, blood vessels, kidneys, lungs, blood pressure, and bowels, the symptoms it causes can affect many areas of the body.

Scleroderma affects the skin to cause local or widespread signs of inflammation (redness, swelling, tenderness, itching, and pain) that can lead to skin tightness or hardening. These skin changes can be widespread, but it's most common for them to affect the fingers, feet, face, and neck. This can lead to decreased range of motion of the fingers, toes, and jaw. Tiny areas of calcification (calcinosis), while not common, can sometimes be noticed as hard nodules at the tips of the elbows, knees, in the fingers, or over joints.

Scleroderma commonly affects the esophagus leading to heartburn. This is directly a result of stomach acid flowing back up into the esophagus. Sometimes this can lead to scarring of the esophagus, resulting in narrowing with difficulty swallowing food and/or localized pain in the central chest.

Blood vessels that can be affected include the tiny arterioles of the finger tips, toes, and elsewhere. These vessels can have a tendency to spasm when the areas are exposed to cold, leading to blueness, whiteness, and redness of involved fingers, toes, and sometimes nose or ears. These color changes are referred to as Raynaud's phenomenon. Raynaud's phenomenon may precede the development of other features of scleroderma by years. It should be noted that Raynaud's phenomenon can be an isolated disease without any other disease such as scleroderma developing. Raynaud's phenomenon can cause inadequate supply of oxygen to the involved tips of fingers or toes, causing tiny ulcers or blackened (dead) skin. Sometimes Raynaud's phenomenon is also associated with tingling. Other blood vessels that can be involved in scleroderma are the tiny capillaries of the face, lips, mouth, and fingers. These capillaries widen (dilate), forming tiny, red blanching spots, called telangiectasias. Rarely, telangiectasias in the stomach (called "watermelon stomach" because of its appearance) can present a serious risk of bleeding and require close monitoring by gastroenterologists.

Elevated blood pressure is potentially serious and can lead to kidney damage (renal crisis). Symptoms include headache, fatigue, and in severe cases, stroke. Blood pressure monitoring and control is essential.

Inflammation of the lungs in scleroderma can cause scarring, resulting in shortness of breath, especially with physical exertion. Elevated pressure in the arteries to the lungs (pulmonary hypertension) can also cause shortness of breath and difficulty getting an adequate breath with activity.

Scleroderma involvement in the heart can lead to damage to heart muscle, irregular heart rhythms, abnormal electrical activity of the heart, and inflammation around the heart (pericarditis).

Scleroderma affecting the large bowel (colon) most often causes constipation but can also lead to cramping and diarrhea. When this is severe, complete stool blockage (fecal impaction) can result.

Medically Reviewed by a Doctor on 12/10/2014

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