What is systemic scleroderma?

Scleroderma, also known as systemic sclerosis, is a clinical condition characterized by limited or diffuse skin thickening and fibrosis. Learn more about the causes, typology and treatment of systemic scleroderma: Causes of systemic scleroderma While limited scleroderma generally causes damage to organs, systemic scleroderma may lead to organ damage, organ insufficiency and organ failure, which may be life-threatening in severe cases. Therefore, systemic scleroderma is generally considered more difficult to be dangerous and more difficult to treat than limited scleroderma, and the prognosis is poor because of the potential for organ damage. As with limited scleroderma, it is not yet possible to medically explain the rationale for the development of systemic scleroderma, but at this stage it is generally accepted that the onset of the disease is related to genetic and environmental factors. The use of drugs such as bleomycin, the involvement of H. pylori, polyvinyl chloride, organic solvents, silicon, silica, and epoxy resins are all suspected to be associated with the development of systemic scleroderma. People with long-term exposure to similar drugs or agents should be alerted to similar environmental factors. The subtypes of systemic scleroderma There are also two subtypes of systemic scleroderma, diffuse scleroderma and acrodermic scleroderma. Disseminated scleroderma is the more common and the more damaging one to patients. It manifests as symmetrical tightening, hardening, and thickening of the skin on the extremities and face, and may involve the proximal extremities and the neck and trunk, along with damage to the gastrointestinal tract, kidneys, lungs, heart, and other internal organs. This type is characterized by rapid onset, rapid progression of skin damage, early appearance of visceral damage, and poor prognosis. Limbic scleroderma, on the other hand, has a better prognosis, with a 10-year survival rate of about 70%. In this subtype, skin changes are limited to the distal extremities such as the fingers, forearms, distal lower extremities and face, with symmetrical tightening, hardening and thickening of the skin in these areas. Raynaud’s phenomenon means that the skin of the patient becomes pale after the onset of the disease, then turns from pale to cyanotic, then from cyanotic to flushed, accompanied by coldness, stiffness, difficulty in movement or pins and needles pain in the fingers. This phenomenon can heal spontaneously without intervention, but the discomfort and fear it brings to the patient are stronger. In addition, the presence of Raynaud’s phenomenon generally predicts the onset of systemic scleroderma. Therefore, patients who already have the onset of Raynaud’s phenomenon should be taken seriously. Treatment of systemic scleroderma There are no specific drugs for systemic scleroderma because the pathogenesis is not known. Early treatment is aimed at preventing new skin and organ involvement, while late treatment is aimed at improving existing symptoms. Intervention with drugs such as glucocorticoids and immunosuppressants, penicillamine, etc., can generally be used with good results. Patients should avoid smoking and alcohol when Raynaud’s phenomenon occurs, pay attention to cold and warmth, and reduce the possibility of skin trauma, all of which can help prevent and slow down the onset of systemic scleroderma.