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Description
An in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.
Alternative Names
Systemic sclerosis
Symptoms
Raynaud's Phenomenon
Raynaud's phenomenon is often the first sign of the scleroderma disease process. With this condition, small blood vessels narrow in the fingers, toes, ears, and even the nose.
Typically, the fingers go through three color changes:
- First, they become very pale.
- As the blood flow is cut off, they turn a bluish color, usually in the top two sections of the second and third fingers.
- Finally, when blood flow returns, the fingers become red.
- Tingling and pain may occur in the affected regions.
Attacks of Raynaud's phenomenon can occur several times a day, and are often brought on or made worse by cold. Warmth relieves these attacks. In severe cases, attacks may develop regardless of the temperature. Severe cases may also cause open sores or damage to the skin and bones, if the circulation is cut off for too long. The syndrome may also be triggered by stress.
It is important to note that over 80% of cases of Raynaud's phenomenon are harmless. It is very common and occurs in 3 - 5% of the general population. This condition is more likely to be a symptom of scleroderma or some other connective tissue disease if it develops after age 30, if it is severe, and if it is accompanied by other symptoms (such as skin changes and arthritis).
Skin Changes
Course of Typical Skin Changes. The primary symptoms of scleroderma occur in the skin . They often take the following course:
- Typically, pitted scars appear first on the hands. The skin begins to thicken and harden on the hands, feet, and face. The fingers may swell. This condition is called sclerodactylia or acrosclerosis. Patients with diffuse scleroderma may have whole hand swelling before the skin significantly thickens.
- Thickened or hardened patches on other areas of the body may also develop. (Their appearance on the trunk and near the elbows or knees tends to be a sign of a more severe condition.)
- For the first 2 or 3 years, the skin continues to thicken and feel puffy.
- This process then stops, and can even get better. The skin may soften.
- As the disease progresses further, however, the skin loses its ability to stretch, and becomes shiny as it tightens across the underlying bone, particularly in the fingers, toes, and around the mouth.
- Eventually, in severe cases, fingers may lose the ability to move, and can be difficult to bend. The hands and feet may curl from the tightness of the skin. It may be difficult to open the mouth widely.
Other Skin Changes. The following skin symptoms may also occur:
- Flat red marks, known as telangiectasis , may appear in various locations, usually the face, palms, lips, or the inside of the mouth.
- In calcinosis , small white lumps form beneath the skin, sometimes oozing a white substance that looks like toothpaste. Calcinosis can lead to infections.
- The base of the fingernails may show loss of small blood vessels in some places, and widening of small blood vessels in others. This is an indication that internal organs might be involved.
- The entire surface of the skin may get darker over time, and contain patches of abnormally pale skin.
- Hair loss may occur.
- About 1% of patients have Sjogren syndrome , a group of symptoms that include dry eyes and dry mucous membranes (such as those in the mouth).
- Inside the mouth, scleroderma can also cause changes that impair gum healing.
Bone and Muscle Symptoms
Changes in bones, joints, and muscles may cause the following symptoms:
- Mild arthritis. The condition is usually distributed equally on both sides of the body.
- Bone loss in the fingers. The destruction is not as severe as it is in rheumatoid arthritis, although the fingers may shorten over time.
- Trouble bending the fingers, if the disease has affected the tendons and joints.
- Muscles weakness may occur, especially near the shoulder and hip.
Symptoms in the Digestive Tract
The development of digestive symptoms depend on the extent of the condition:
- Symptoms of gastroesophageal reflux disorder (heartburn and difficulty in swallowing) are common in the upper part of the digestive tract.
- If the lower part of the digestive tract is affected, patients may become constipated. If scarring in the lower intestine is very severe, watery diarrhea may develop. Many patients do not have lower digestive tract symptoms, although the disease commonly affects this region.
Symptoms in the Lungs
In severe cases, the lungs may be affected, causing shortness of breath or difficulty in taking deep breaths. Shortness of breath may be a symptom of pulmonary hypertension, an uncommon but life-threatening complication of systemic scleroderma.
- Review Date: 12/14/2006
- Reviewed By: Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital
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