Diffuse systemic scleroderma is a subtype of systemic scleroderma. It is evident by more or wider areas of thickened skin and it is characterized by an early internal inflammatory phase. Unlike limited scleroderma, the onset of the diffuse systemic symptoms may be rapid, severe, and can greatly impact quality of life.
Patients with diffuse systemic scleroderma are more likely to have extensive skin fibrosis on the arms, legs, and trunk — and a higher risk of developing systemic sclerosis (fibrous hardening of the internal organs).
Tightening of the skin makes it difficult for patients to bend fingers, hands, and other joints. The skin can lose or gain pigment, become swollen, and have chronic itchiness. Sometimes joints, tendons, and muscles can become inflamed and painful. Internal organs such as the gastrointestinal tract, heart, lungs, or kidneys may become damaged. Symptom severity can differ patient by patient.
Problems with the gastrointestinal tract may bring difficulties with eating, food absorption, and elimination. Pulmonary arterial hypertension is the leading cause of death among people with scleroderma.
People with diffuse scleroderma are often tired, have a diminished appetite, and experience weight loss. Sometimes people with the disease enter a phase where disease progression stabilizes. This phase usually begins three to five years after diagnosis and last for different lengths of time from patient to patient.
There is currently no cure for diffuse scleroderma, but treatments are available to ease symptoms. Although the disease may lie dormant for several years, there is no certainty whether symptoms will improve, remain the same, or worsen.
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