Scleroderma (progressive systemic sclerosis) is a chronic disease of unknown etiology, characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, articular structures and other organs (kidneys, lung, heart, gastrointestinal and skeletal muscles). The majority of manifestations have vascular features (e.g., Raynaud's phenomenon), but frank vasculitis is rarely seen. It can range from a mild disease, affecting the skin, to a systemic disease that can cause death in a few months.
Scleroderma predominantly affects women (Female:Male = 4:1). Symptoms usually appear in the 3rd to 5th decade.
The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmobility, sclerodactyly, telangiectasia) often occurs. Associated syndromes include Rheumatoid arthritis, Systemic lupus erythematosus, and Polymyositis.
Treatment is symptomatic and supportive. Dialysis may be necessary as the disease progresses.
The cause of scleroderms is unknown. Several mechanisms have been proposed, including alterations in immune response. There are some association with quartz mining, quarrying, vinyl chloride, and hydrocarbons. Treatment with bleomycin (an antibiotic which splits single and double-stranded DNA) has caused a scleroderma-like syndrome.
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