Scleroderma: what it is, symptoms, causes, treatment

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scleroderma It is a disease that affects the connective tissue, promoting hardening and/or thickening of the skin and fibrosis of the tissues involved. Scleroderma can be classified into two main types: localized and systemic. The localized form affects the skin and spares the internal organs, while the systemic form affects the internal organs.

The localized form is more common in children, while the systemic form occurs mainly in the fourth decade of life. The diagnosis is made by analyzing symptoms and carrying out complementary tests. There is no cure for scleroderma.

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Topics of this article

  • 1 - Summary about scleroderma
  • 2 - What is scleroderma?
  • 3 - Localized and systemic scleroderma
  • 4 - Symptoms of scleroderma
  • 5 - Diagnosis of scleroderma
  • 6 - Treatment of scleroderma

summary about scleroderma

  • Scleroderma affects the connective tissue.

  • The disease affects the skin and can also affect organs internal.

  • It can be classified into localized and systemic.

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  • There are no treatments to cure it, but there are those that prevent its progression.

What is scleroderma?

Scleroderma is a illness whatand affects connective tissue and is characterized by hardening and/or thickening of the skin and fibrosis of the tissues involved. The disease takes place in the hardening of the connective tissue, which becomes scarring and fibrotic. The term derives from the Greek sclerus, which means “hard”, and derma, which means “skin”. The etiology of the disease is unknown, but it has been associated with problems such as infections, trauma, vaccination and autoimmune diseases.

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Localized and systemic scleroderma

Scleroderma can be of two types: localized and systemic. A localized scleroderma affects the skin and subcutaneous tissues, sparing the internal organs. This form usually occurs at school age.

Localized scleroderma can be subclassified into: morphea and linear. Morphea is the most common form and presents as areas of thickened skin, usually oval in shape and more frequently on the trunk, although it also appears on the arms, legs or forehead. The linear form presents itself as areas in strips or bands of hardened skin that appear on the trunk or limbs. It receives the denomination “in coup de saber” when it appears in the frontal region.

In systemic scleroderma, also called systemic sclerosis, in turn, in addition to the skin, internal organs are also affected. According to the Brazilian Society of Rheumatology, the systemic form is four times more frequent in females than in males and affects mainly in the fourth decade of life.

Systemic scleroderma can be subdivided into limited and diffuse. In the diffuse one, there is greater involvement of internal organs, while the limited one presents a later and confined involvement of internal organs.

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symptoms of scleroderma

The clinical manifestations of scleroderma depend on the type that the person has. In localized scleroderma, symptoms range from small plaques to aesthetic and functional deformities. In its milder forms, only slight atrophy of the affected region is observed, while, in more severe forms, deeper tissues are affected, such as subcutaneous tissue, muscles and bones. As it mainly affects children, one of the possible complications of this scleroderma is the delay in bone growth.

With regard to systemic scleroderma, the symptoms are nonspecific, which may include tiredness, muscle and skeletal complaints, swelling of the hands, gastroesophageal reflux, dysphagia, shortness of breath, increased blood pressure, palpitations and Raynaud's phenomenon. Raynaud's phenomenon causes changes in the color of the body's extremities, which may become pale or bluish when exposed to low temperatures, or reddish when heated.

Fingers in different colors, illustrating Raynaud's phenomenon.
Raynaud's phenomenon can occur in case of scleroderma and is characterized by changes in the color of the body's extremities.

Diagnosis of scleroderma

The diagnosis of scleroderma is made based on the analysis of the symptoms presented by the individual as well as the performance of tests. Some of the tests that can be done are: nailfold capillaroscopy, blood tests and biopsies.

treatment of scleroderma

The treatment varies between localized and systemic scleroderma. The first is a disease that has no cure, and its treatments seek to alter its progress. Among the measures adopted are phototherapy, use of oral and topical medications, as well as physiotherapy. According to the Brazilian Society of Rheumatology, this type of scleroderma, in general, presents a self-limited course, becoming inactive and with spontaneous improvement from three to five years.

The second also has no cure. Depending on the organ affected by the disease, different medications may be recommended. For the treatment of Raynaud's phenomenon, vasodilator drugs are indicated, as well as it is recommended to avoid washing hands with cold water and warming the extremities with gloves and socks.

By Vanessa Sardinha dos Santos
Biology teacher

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