Symptoms
Symptoms of localized scleroderma develop slowly. Diffuse and limited scleroderma symptoms may progress slowly or very rapidly.
Raynauds Phenomenon
Raynauds phenomenon is often the first manifestation of the scleroderma disease process. With this condition, small blood vessels constrict usually the arteries in the fingers, toes, ears, and even the nose.
Typically, the fingers undergo three color changes.
- First, they become very pale.
- As the circulation is cut off, they turn a bluish color, usually in the top two sections of the second and third fingers.
- Finally, when blood returns the fingers become red.
- Tingling and pain may occur in the affected regions.
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Click the icon to see an image of raynaud's phenomenon. |
Attacks can occur several times a day and are often triggered or exacerbated by cold and relieved by warmth. In severe cases, cold may have little influence on whether the attacks occur or not. The syndrome may also be triggered by stress. In severe cases, if the circulation is cut off for prolonged periods, ulcers or even gangrene may develop.
It is important to note over 80% of cases of Raynauds phenomenon are harmless. It is very common and occurs in 3% o 5% of the general population. This condition is more likely to be associated with 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 (e.g., skin changes, arthritis).
Skin Changes
Course of Typical Skin Changes and Hardening. 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. (The 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 indicate a more severe condition.)
- For the first two or three years, the skin continues to thicken and to feel puffy.
- Then this process stops and can even recede. The skin may soften.
- As the disease progresses further, however, the skin loses its elasticity and becomes shiny as it stretches across the underlying bone, particularly in the fingers, toes, and around the mouth.
- Eventually, in severe cases, fingers may lose mobility and be difficult to bend. The hands and feet may curl from the tautness of the skin. It may be difficult to open the mouth widely.
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Click the icon to see an image of sclerodactyly. |
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 inside the mouth.
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Click the icon to see an image of telangiectasia. |
- In calcinosis, small white lumps form beneath the skin, sometimes oozing a white substance resembling toothpaste, which can lead to infections.
- The base of the fingernails may show capillary (small blood vessel) loss in some places and capillary dilation in others, an indication that organ involvement may be present.
- The entire surface of the skin may darken over time and contain patches of abnormally pale skin.
- Hair loss may occur.
- About one percent of patients have Sjogrens 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 gum changes that impair healing.
Bone and Muscular Symptoms
Changes in bones, joints, and muscles may cause the following symptoms:
- Mild arthritis usually distributed symmetrically 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, especially near the shoulder and hip.
Symptoms in the Gastrointestinal Tract
The development of gastrointestinal (GI) symptoms depend on the extent of the condition:
- Symptoms of gastroesophageal reflux disorder (heartburn and difficulty in swallowing) are common in the upper GI tract.
- If the lower GI tract is affected, patients may become constipated. If scarring in the lower intestine is very severe, watery diarrhea may develop. Many patients have few or even no lower gastrointestinal 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.)
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Click the icon to see an image of the respiratory system. |
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