Scleroderma: Types, Symptoms, Causes and Treatment

It is one of the rare diseases

Scleroderma groups a set of rare diseases that affect the skin, narrowing and hardening their connective tissues. In some people, scleroderma affects only the skin.

However, in many people, scleroderma damages structures that are beyond the skin, such as internal organs, blood vessels, and the digestive tract. The signs and symptoms vary, depending on the structures that are affected.

This disease affects women more often than men and most commonly occurs between the ages of 30 and 50. While there is no cure for scleroderma, a variety of treatments can relieve symptoms and greatly improve the quality of the disease. life of those who suffer from this disease.

According to the Scleroderma Foundation, scleroderma is a chronic systemic autoimmune disease characterized by hardening (“sclero”) of the skin (“derma”), although it can also affect the internal organs.

Currently there is no cure for scleroderma, but there are effective treatments for some forms of the disease and its wide range of symptoms.

The six types of scleroderma can be separated into two main classifications: localized scleroderma and systemic sclerosis. However, patients may have different symptoms and different combinations of diseases.

There have been many attempts to classify scleroderma so that patients and doctors can plan for the proper management of the condition.

This is because the condition varies widely in different patients, with some having only a very small problem with any progression of the disorder, while others may have a very serious illness.

Fortunately, most patients have the mildest disease. Basically there are two types of scleroderma:

  1. The first is called limited
  2. The second diffuse.

The degree of skin involvement is used to divide patients into these two groups. Limited scleroderma usually causes Raynaud’s phenomena and hardening of the skin on the hands.

Types

Localized scleroderma

Localized scleroderma is characterized by inflammation and thickening of the skin by excessive deposition of collagen.

Relatively mild, only a few areas of the body are affected – usually the skin or muscles. Localized scleroderma rarely becomes systemic, internal organs are usually not affected.

Cutaneous

Cutaneous scleroderma is a subtype of systemic sclerosis characterized by the association of Raynaud’s phenomenon and fibrosis of the skin on the hands, face, feet and forearms.

The exact cause is unknown, but probably originates from an autoimmune reaction that leads to the overproduction of collagen. In some cases, the condition is associated with exposure to certain chemicals. The administration is aimed at treating the symptoms present in each affected individual.

Morfea

Classified as a form of localized scleroderma, it usually involves isolated patches of hardened skin of different sizes, shapes and colors that can disappear and return at any time. There is no involvement of internal organs.

Linear scleroderma

This type of localized scleroderma is usually characterized by a thickened skin line that can affect the bones and muscles beneath it.

It most commonly appears on the arms, legs, or forehead, although it is possible that linear scleroderma occurs in other areas. It usually appears in childhood, linear scleroderma can affect the growth of the extremities.

Systemic sclerosis

Systemic sclerosis is a multi-system autoimmune disease that produces fibrosis (development of scar tissue), vascular damage and abnormal growth of connective tissue in many parts of the body including muscles and joints.

These abnormalities lead to the breakdown of skin, subcutaneous tissue, muscles and internal organs such as the heart, lungs, kidneys and intestinal tract.

Diffuse scleroderma

In diffuse scleroderma, more areas of the skin swell. It is more likely that the skin of the arms, legs and trunk is involved.

Patients with diffuse scleroderma have an increased risk of developing sclerosis or fibrous hardening of the internal organs. In this case, the thickening of the skin occurs rapidly.

This means that there are more areas of skin involved and thickened, but there is a high degree of variability among patients.

Tight skin makes it difficult to bend fingers, hands and other joints. Sometimes there is inflammation of the joints, tendons and muscles. Tight skin on the face can reduce the size of a person’s mouth and make good dental care a very complicated task.

The skin can lose or gain pigment; Coloring skin areas of light or dark tones. Some people lose hair on the tips, sweat less, and develop dry skin due to skin damage.

Diffuse scleroderma can have an associated involvement of internal organs such as the gastrointestinal tract, heart, lungs or kidneys. The degree of involvement of the organs is very variable: some do not receive any damage and other organs of the patient can be seriously affected.

Scleroderma limited

About half of all patients with scleroderma have this slower and less harmful form of scleroderma. Thickening of the skin is less widespread and typically confined to the fingers, hands and face.

In some cases, limited scleroderma can also affect the digestive tract, lungs or heart. In general, it develops slowly over the years.

Limited scleroderma is also known as CREST syndrome. “CREST” (in English) is the acronym for the following conditions that occur in limited sclerosis: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly and elangiectasia.

Limited scleroderma means that only limited areas of the skin are thick; Usually only the fingers and / or the face. Limited scleroderma is the mildest form of scleroderma.

It is more common among Caucasians than other populations. Each person with scleroderma is different and has a different pattern of symptoms. CREST syndrome is a type of limited scleroderma. CREST (in English) means the following:

  • C (calcinosis) is for calcium deposits under the skin and in tissues.
  • R means the Raynaud phenomenon.
  • E is for esophageal dysmotility. This causes heartburn, which is often experienced by CREST patients.
  • S is for sclerodactyly; Which means thick skin on the fingers.
  • T is for telangiectasias, which are enlarged blood vessels. These appear as red spots on the face and other areas.

Calcium in the skin and tissues can be a painful discomfort that can irritate or break the surface of the skin. Raynaud’s attacks can be uncomfortable and associated with cold intolerance. Acid reflux of esophageal dysmotility can be painful causing irritation in the lining of the esophagus.

Skin changes in limited scleroderma usually do not progress. Telangiectasia is not harmful and can be eliminated by laser therapy. In general, limited scleroderma causes less involvement of the organs of the body than the more severe form.

Some patients may develop lung and heart disease. Doctors at the Johns Hopkins Scleroderma Center in the United States offer treatment options that can provide relief to patients and prevent complications.

symptom

The symptoms of this disease vary from person to person, everything depends on the parts of the body that are affected.

– The digestive system: In addition to causing reflux, which can affect the section of the esophagus that connects to the stomach, some people may also have problems absorbing nutrients from food if the intestinal muscles do not move food correctly through the intestines.

– The fingers: The exaggerated response to cold that causes numbness, changes in color and pain in the fingers.

– The heart, kidneys and lungs: Rarely affects the function of the heart, kidneys or lungs. These problems can pose a great risk to the patient’s life.

– The skin: The majority of patients suffering from scleroderma experience a hardening and narrowing of skin spots. The spots can have oval or straight lines.

The amount of these spots varies according to the type of Scleroderma, in many of the patients the skin can take a bright tone, this due to the swelling.

What causes scleroderma?

This disease is the result of overproduction and accumulation of collagen in all tissues of the body. Although doctors still do not know for sure why the body drives this abnormal production of collagen, the body’s immune system seems to play an important role.

For reasons that are still unknown, the immune system attacks all the cells in the body, causing inflammation and overproduction of collagen.

Are there risk factors?

This disease occurs more frequently in women than in men. Native Americans and the African-American population are more likely than ascending Americans of Europeans to develop scleroderma that attacks internal organs.

Is there any treatment or cure?

Only in some cases, the symptoms of this disease disappear in about three to five years. Generally, the variant of scleroderma that attacks internal organs does vary and gets worse over time.

To date, no medication has been developed that can stop the process of scleroderma, which is the excessive production of collagen. Even so, doctors work to find solutions that alleviate symptoms and improve the quality of life of patients.

Currently therapy is performed with medications indicated for blood pressure, which work by dilating blood vessels and preventing long-term kidney and lung problems. Similarly, doctors also prescribe medications to suppress the immune system, such as those taken by people after organ transplants.

To reduce reflux and prevent stomach acids from damaging the walls of the stomach, doctors prescribe omeprazole, which can relieve your symptoms.