Scleroderma is an autoimmune disease of the group of collagenases. It is a chronic generalized disease associated with inflammation and connective tissue proliferation. Skin and blood cells, but also the lungs, heart, and digestive tract may be affected.
A distinction is made between the more common limited systemic sclerosis and the less common diffuse systemic sclerosis. In the case of the limited form, sclerosis affects especially the face and the acres, an organ infestation only occurs later. The diffuse form is characterized by a much more aggressive course with a generalized involvement of the skin and an early involvement of the organ. The first sign of the disease is usually Raynaud's syndrome, where certain causes, such as cold or stress, result in painful whitening of the fingers with subsequent reactive hyperemia. There is often a motility of the esophagus with concomitant gastroesophageal reflux and dysphagia. Possible complications may include pulmonary fibrosis, pulmonary hypertension, and cardiomyopathy.
The exact cause of scleroderma is unknown. Genetic predisposition and chronic chemical exposure (silica, solvents, hydrocarbons) are discussed as being causative. Women are about four times more likely than men to be affected. The disease usually manifests between the ages of 40 and 50 years.
The limited-form prognosis is relatively good (10-year survival 80-90%), which is much worse for diffuse form because of the increased risk of life-threatening complications.