Scleroderma: The disease and its symptoms

The immune system of the body acts as a shield against the attack of the all external germs and infections. But what happens if this shield becomes the very weapon? Scleroderma is such an autoimmune disease, where the body’s immune system attacks its own healthy tissues. This is a disease which affects the body’s connective tissue.

As the name suggests, Scleroderma affects the skin but its impact penetrates to other organs as well. Inflammation of skin, with redness, itching, swelling, tenderness of the area and even pain are the first symptoms to look out for. This is followed by scar tissue formation, also called fibrosis, on skin and other organs. This scarring causes the affected areas to thicken and become firm.

Scleroderma affects multiple organs of the body but it is the extent of affected skin area which helps us to categorize the disease. When the disease affects the body in a widespread or diffuse fashion, it is termed Systemic sclerosis. This causes the skin of the body extremities, trunk- including chest, back, flanks or the abdomen, and face to harden with rapidity, progressing after an initial inflammatory stage. The thickening in this type of Scleroderma is usually symmetric and organ disease involvement is rapid and often, serious.

The organs which are affected are – lungs, where tissue scarring or fibrosis occurs, bowels, esophagus, kidneys and heart. It is often accompanied by high blood pressure which can lead to a multitude of complications.

Kidneys – In the kidneys, the extent of the disease causes severe damage of blood vessels, and can further elevate the blood pressure leading to the swelling of brain, accompanied headaches and can even cause a stroke.

Heart – High blood pressure pressurizes the heart as well, causing heart rhythm to fluctuate and over time, can lead to enlargement of the heart – all leading to a heart attack.

Lungs – In lungs, Scleroderma causes inflammation, which in turn causes healthy lung tissue to scar. A condition called pulmonary hypertension can also develop which increases pressure in the arteries which bring blood to the lungs. This also causes severe breathing stress, which increases with increased physical exertion.

Esophagus – Damage or scarring of the esophagus can show a lot of symptoms, but the most common being chronic acidity or acid reflux, where acid in the stomach gets pushed back into the esophagus. Swallowing of food may be difficult and painful. The pain can also be localized in the chest cavity.

The other form of Scleroderma is more localized and usually affects only the skin of the face, fingers and toes. Decreased motor function of the affected areas like the fingers, toes or the jaw is also a symptom. The associated diseases of other organs are slow in their manifestation. This type of Scleroderma is also known as CREST, which stands for five different components of the disease, as seen in most patients. But most patients do not report having all the symptoms at one time.

Calcinosis – This is the first component of CREST, where white or ivory colored areas form on the skin, commonly in the knees, fingers or elbows. These are calcium deposits, which are often tender and susceptible to infection. Infected areas can fall off or have to be surgically removed. As severe as it sounds, it is also the most rarely seen symptom of scleroderma.

Raynaud’s phenomenon- This is the second component, where blood vessels or arteries carrying blood to the ears, tongue, fingers, toes and nose become prone to frequent spasms which in turn cause a change of color when exposed to cold, heat or even emotional turmoil. These areas change from a blue to white and finally, red color after exposure. This phenomenon can hamper the supply of oxygen to the tips of the fingers and toes, causing an outbreak of tiny black spots or ulcers. It can also cause a tingling sensation to the affected parts.

Esophagus- This is often affected in this type of Scleroderma and is the third component. The disease makes the muscles of the esophagus sluggish and the esophagus becomes unusually wide which causes acid reflux.

Sclerodactyly – This is the fourth component and is characterized by the tightness and thickening of the skin of toes or fingers. This gives an appearance of puffiness and the skin seems ‘shiny’. This causes severe motor disability but progresses slower than Diffuse Scleroderma.

Telengiectasias - Capillaries of the fingers, face, mouth and lips can also be affected, causing them to widen which forms small, red spots and this is the last component of CREST. These red areas whiten when pressed.

While Scleroderma can be classified into two general groups of Systemic Sclerosis and the CREST variant, patients can exhibit symptoms which are a combination of both variants of the disease

Sometimes the disease can also occur in localized form, called Morphea. This variant is not accompanied by any other disease but that of the skin. Affected areas become black and patchy and may occur as multiple lesions across the skin.

It is important that a doctor be consulted without any delay if any of the symptoms are noticed.

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