Scleroderma is an autoimmune disease that affects connective tissues. The problem with this disorder is that it leads to damage and fibrosis of tissues. A recent study has discovered new culprits that may be responsible for this disorder.
What is Scleroderma?
Scleroderma is the dermatologic (skin) component of the manifestations of systemic sclerosis. Systemic sclerosis, on the other hand, is a multisystem autoimmune disease wherein there is increased fibroblastic activity, thus leading to abnormal growth of connective tissue. This disorder can cause vascular damage and fibrosis which may occur in the skin, known as scleroderma.
There are two main types of scleroderma: limited cutaneous systemic sclerosis (lcSSc) or limited scleroderma and diffuse cutaneous systemic sclerosis (dcSSc) or diffuse scleroderma. Limited cutaneous systemic sclerosis usually consists of 70% of systemic sclerosis cases and usually affects only the face, the forearms and the lower legs. This type may also be known as CREST syndrome which stands for Calcinosis, Raynaud’s disease, Esophageal dysmotility, Sclerodactyly and Telangiectasia. On the other hand, diffuse scleroderma consists of only 30% of systemic sclerosis cases and involves the upper arms, the thighs or the trunk. There are also other types of scleroderma such as systemic sclerosis sine scleroderma, in which there is internal organ involvement without the skin changes.
Scleroderma commonly affects women who are within 30 to 50 years of age, though it may affect men and people of all ages. The etiology of this disease is still unknown. The features of this medical condition are excessive collage production, excessive collagen deposition, inflammation, vascular damage and autoimmunity. There is probably a genetic component in scleroderma. Some experts say that the causes may include infection with viruses such as cytomegalovirus, chemicals such as pesticides or benzene derivatives or silica, drugs such as weight loss pills or penicillamine or cocaine.
Scleroderma may manifest itself as Raynaud’s phenomenon, hardening of the skin in the hands or face, and esophageal symptoms. There may also be non-specific signs and symptoms such as musculoskeletal pain, fatigue, and hand swelling. Internal organs may also be involved however the severity of skin involvement is not often [predicative of the severity of internal organ involvement.
Limited cutaneous systemic sclerosis is usually milder than the other types and may have a slow onset and slow progression. This may go on until there are internal complications that may occur. On the other hand, diffuse cutaneous systemic sclerosis may have a more rapid onset and may bring about skin thickening and Raynaud’s phenomenon. The skin changes may appear rapidly and may gradually disappear with softening of skin changes and improvement in mobility. The symptoms may be worse during the first 3 to 5 days and may soon stabilize. There may be internal organ involvement.
Symptoms of scleroderma include fatigue, weight loss, swelling of the fingers and toes, sausage-like appearance of the digits, hardening and thickening of the skin, reduced hand movements, pitted fingertips, calcinosis, telangiectasia, joint pain and swelling, gastrointestinal symptoms and pulmonary symptoms.
Newly Discovered Causes of Scleroderma
A recent study by researchers from Northwestern Medicine has identified potential drug treatment targets for scleroderma. This study found out that a damage-associated protein called fibronectin (FnEDA) present in scleroderma may be the culprit in immune response that convert tissue repair into chronic fibrosis. FnEDA is undetectable in normal adults but it is markedly increased in the skin biopsies of patients with scleroderma. The results were published in the April 16 issue of Science Translational Medicine.
The scientists were able to investigate the connection of immunity and fibrosis with scleroderma by looking at skin biopsies of scleroderma patients. They also sought to identify the factors responsible for scarring in scleroderma as well as to test the theory that FnEDA was needed for the scarring to occur.
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