Scleroderma is a condition in which a type of connective tissue in the body called collagen is overproduced. It can be localized, leading to hardened patches or bands of skin, the tissue that lies immediately below the skin and sometimes the muscles of the arms, legs or forehead. It can also be systemic--sometimes called systemic sclerosis--in which case the excess collagen has a more widespread effect. Systemic scleroderma can be further divided into limited or diffuse categories; the NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases adds a third category: systemic sclerosis sine, which does not feature the more overt skin changes.
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For all types of sclerosis, Raynaud’s disease is the most common--and frequently the first--symptom, afflicting more than 90 percent of those with scleroderma, according to the Arthritis Foundation. This disease, also known as Raynaud’s phenomenon, occurs when the tiny capillary blood vessels in the fingers and toes constrict due to cold temperatures or emotional distress. Toes or fingertips will quickly turn white, then blue; this usually lasts up to 15 minutes. The Mayo Clinic says that the type of Raynaud's phenomenon associated with scleroderma may cause permanent damage to skin on the fingertips.
This acronym refers to the group of symptoms that may be found to some degree in most cases of limited cutaneous scleroderma, which generally affects only the arms, fingers, legs, face and neck. CREST symptoms are calcinosis, Raynaud's, esophagus, sclerodactyly, telangiectasias. Calcinosis refers to calcium deposits in the joints; esophagus refers to difficulty in swallowing; sclerodactyly refers to thickening and tightening of the skin of the fingers; telangiectasias refers to tiny red spots on the hands and face resulting from swollen blood vessels. Not all of these are seen in the early stages of scleroderma, and Raynaud’s may be the only early symptom of limited cutaneous scleroderma.
While thickening of the skin due to the overproduction of collagen is a hallmark feature of scleroderma, the speed with which it occurs varies depending on the type of scleroderma. In the limited form of the disease, it may not appear for many years. In diffuse systemic scleroderma, it comes on suddenly, starting with the hands, then spreading to the face, chest, stomach and upper arms and legs, and affecting both sides of the body. Early on, skin may be red, swollen and inflamed. The joints, especially in the hands, may feel stiff and painful.
Other Possible Early Symptoms
If scleroderma affects the organs, early signs and symptoms may include gastrointestinal problems, such as reflux and bloating, and shortness of breath if the disease has reached the respiratory system. Those with the diffuse form of the disease may also experience fatigue, depression and loss of appetite leading to malnutrition and weight loss.