Diffuse scleroderma

Diffuse systemic sclerosis

Overview

Type of disease: Rare conditions

Diffuse scleroderma is a type of scleroderma characterized as a systemic (internal) chronic connective tissue disease that is visible through the hardening of the skin (please also see: Scleroderma).

Scleroderma can be diffuse or limited. In diffuse scleroderma, skin thickening and hardening occurs more rapidly and involves more skin areas than in limited disease. Skin of the arms, legs, and trunk are more likely to be involved. The tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes inflammation of the joints, tendons and muscles. Tight skin on the face can reduce the size of a person’s mouth and increases the importance of good dental care. Skin may gain or lose pigment (color) creating light and dark patches. In addition, people with diffuse scleroderma have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs such as the heart, lungs and kidneys. The amount of organ involvement is highly variable; some individuals have no organ involvement while others may be severely affected. Scleroderma can develop in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55 and affects females more frequently than males.

The diagnostic process may require consultation with rheumatologists (arthritis specialists), and/or dermatologists (skin specialists) and require blood studies and numerous other specialized tests. Specific organ and skin treatments are available. Treatments range from anti-inflammatory medications, immunosuppressive therapy, drug therapy of vascular disease, and anti-fibrotic agents. If you or a loved one has been diagnosed with diffuse scleroderma, talk to your doctor about the most current treatment options. Support groups are also a good source of information and support.

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