What is Scleroderma?
Scleroderma is a chronic and rare type of autoimmune disease that primarily targets the skin and the body’s connective tissues. The disease involves progressive affectation of the body through gradual hardening and tightening of the skin and connective tissues – components of the body that are naturally meant to be elastic and flexible. The condition mostly affects individuals who are aged anywhere between 30 to 50 years old, and it is more common in females rather than in males. Scleroderma is not contagious but can be a serious challenge for those who suffer from it, especially since it affects the person’s appearance as well as his ability to perform activities of daily living.
There are two forms of scleroderma, Limited Systemic Scleroderma and Diffuse Systemic Scleroderma. The first form is usually limited to symptoms that affect the skin on the hands, arms, and face, or the most superficial layers of the body. It is slow to progress and rarely ever affects the entirety of the body. The second form however is a more serious type of scleroderma. It can progress at a more rapid rate and affects more than just the skin, spreading towards the internal organs such as the kidneys, heart, or lungs. Symptoms associated with the disease depend on the type of scleroderma the person has.
Symptoms of the condition are initially present on the skin or localized areas such as the hands and face. Symptoms include:
- Loss of hair
- Hardening of the skin
- Abnormal lightening or darkening of the skin
- Thickened and stiff fingers, hands, and forearms; along with tightness
- Fingers or toes turn white or blue as a reaction to extreme temperatures (also known as Raynaud’s phenomenon)
- Sores or ulcerations on tips of fingers or toes
- Formation of small bumps on the skin which can contain a white substance
- Skin on face is tightened and mask-like
The bones and muscles may eventually become affected in the condition. Symptoms in these areas are:
- Painful joints
- Pain and numbness of the feet
- Pain and stiffness of the fingers and joints, observably more difficult to move or manipulate
- Pain in the wrist
In advanced cases, the respiratory tract can become affected, impairing the functions of the lungs. This results in the following symptoms:
- Persistent dry cough
- Shortness of breath
Symptoms that involve the digestive tract also occur. These are the following:
- Abdominal bloating after eating
- Heartburn after eating
- Esophageal reflux or burning in the esophageal region due to upsurge of stomach acid
- Difficulty in controlling bowel movements, also known as fecal incontinence
Since scleroderma is chiefly an autoimmune disorder, the most basic root of the illness is caused by the patient’s body itself. An autoimmune condition is a result of the body’s inability to recognize healthy tissue within the body and prompts it to attack its own tissue as well as produce antibodies against it.
Other than its autoimmune nature, not much else is known about what in particular causes the disease. The condition is genetically transmittable and can be passed on from parents to offspring.
Scleroderma is essentially the result of an overproduction of collagen within the body. Collagen is an integral protein component of skin and connective tissues, but its overgrowth leads to their thickening and stiffening. The accumulation of the collagen is the body’s autoimmune response, causing the resulting symptoms of the disease. As tissue fibrosis continues over time, small blood vessels can become occluded because of the deposition of collagen in these tight and tiny vessels.
Although doctors aren’t sure what prompts this abnormal collagen production, the body’s immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.
A direct cure is not available because of the lack of information known about the disease, and there is no medication available as of the moment that can halt the overproduction of collagen. Due to the fact that the specific cause that triggers the autoimmune reaction is unknown, treatment plans for scleroderma are merely aimed at relieving any symptoms the patient may experience as well as preventing any complications.
Patients who are dealing with Raynaud’s phenomenon for instance are given medications that increase the flow of blood to the fingers, improving the ability to deal with extreme temperatures. These drugs can incdlue amlodipine, diltiazem, nifedipine, or nicardipine. These medications improve blood vessels dilatation and also help with the prevention of problems in the kidney as well as the lungs that may results from constant deposition of collagen fibers.
The continued development of excessive tissue on the skin is treated through drugs such as colchicine, D-penicillamine, relaxin, and cyclosporine.
The treatment for scleroderma is also largely developed with the intention of addressing the autoimmune nature of the disease. This is done through administering immunosuppressive drugs, such as those given to patients who have just received organ transplants. Some of the drugs used for this purpose are azathioprine, mycophenolate, methotrexate, and cyclophosphamide.
Medications for dilating blood vessels are also given.
Improving pain management and coping with various degrees of loss in the ability to control movements are address through physical or occupational therapy. These help improve the patient’s ability to cope and improve his chances at maintaining independence through performing activities of daily living on his or her own.
Surgery can be indicated in some cases for patients with scleroderma. Amputation can become an option if the patient’s fingers are no longer viable due to the development of gangrene as a result of the lack of blood supply that reaches them. Lung transplants are a possibility for those who have scleroderma and experience extremely high blood pressure in the blood vessels that supply blood to their lungs.
Patients who have limited scleroderma generally have better chances with the illness. These individuals may not develop the life-threatening form of scleroderma. On the other hand, those who have advanced versions of the disease such as in the case of Diffuse systemic scleroderma, the progressive involvement of internal organs can lead to severe complications. Heart failure, kidney failure, and pulmonary hypertension can cause fatalities in those who suffer the illness, lung problems being the most common cause of death in patients with this condition.
At average, 2/3s of the entire number of patients with the condition lives for at least 11 more years after the diagnosis is made. Those who are fortunate to have slow progression cope much better than those who have to suffer quicker worsening of the disease. In addition, the older a patient with scleroderma is, the greater the possibility that they could die from the disease.
Photos, Images and Pictures of Scleroderma…