Diffuse scleroderma

Diffuse scleroderma is a chronic autoimmune condition that primarily affects the skin and internal organs by causing excessive collagen buildup. This leads to thickening and hardening of the skin, often starting on the fingers and spreading to the trunk and face. The disease can also damage organs such as the lungs, heart, and kidneys, which can seriously impact overall health. People with this condition may experience symptoms like skin tightness, difficulty moving joints, and problems with breathing or digestion. The exact cause is unknown, but it involves the immune system attacking the body's own tissues, leading to inflammation and scarring.

Diffuse scleroderma is a systemic autoimmune disorder characterized by widespread fibrosis of the skin and internal organs due to excessive collagen deposition by activated fibroblasts. It is caused by immune dysregulation leading to chronic inflammation, endothelial injury, and fibroblast activation. The disease typically presents with rapid progression of skin thickening proximal to the elbows and knees, often involving the trunk and face. Major clinical significance arises from internal organ involvement, including interstitial lung disease, pulmonary hypertension, renal crisis, and cardiac fibrosis, which contribute to morbidity and mortality. Autoantibodies such as anti-Scl-70 (anti-topoisomerase I) are commonly present and help distinguish it from limited scleroderma. The pathogenesis involves vascular damage, immune activation, and fibroblast proliferation.