Systemic Sclerosis: Warning Symptoms To Pay Attention To

Date February 14, 2018

What is systemic sclerosis?

Systemic sclerosis or systemic scleroderma is an autoimmune disease which targets the connective tissue. The Body produces too much of a particular structural protein called collagen which in turn accumulates in the tissue.

As it is with any autoimmune illness, the immune system attacks its own body which in the case of systemic sclerosis leads to the formation of scar tissue. The name scleroderma (greek - hard skin) comes from the thickening of the skin caused by these scars. Unfortunately, even internal organs can be affected by the scarring which may lead to fatal consequences.

The cause of the disease is still unknown, but it appears to affect women more than men as well as people between the ages of 30 and 55. Systemic sclerosis is not contagious or infectious although it has been linked with an increase in the cancer risk factor. The disease is relatively rare with 300,000 Americans suffering from scleroderma according to the Scleroderma Foundation.

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Types and symptoms

Scleroderma has two distinct types: localized form and the systemic (diffuse) form.

Localized scleroderma

As its name implies, localized scleroderma only appears on specific areas on the skin and muscles and is rarely spread across the body. It is rarely a serious condition since no internal organs are affected and only in sporadic cases do patients with localized scleroderma develop the systemic form. It is divided into four subtypes:


The most common type of localized scleroderma, Morphea is characterized by 3-4 thickened patches of skin sometimes with color disfigurement. Usually painless, it can, however, cause itchiness.

Generalized Morphea

Generalized Morphea covers more skin than Morphea and can, in extreme cases, cover the entire body.

Linear scleroderma

Linear scleroderma is recognized by a discernable line of thickened skin. It can cause severe damage because it often includes the muscles beneath the surface. In some cases, linear scleroderma can appear on the legs and run so deeply that it renders the joints unusable.

En Coup de Sabre

The name of this subtype is the French phrase "cut from a saber." It got its name because of the marks it leaves resemble a sword cut. It can have catastrophic consequences as it leads to tissue loss (atrophy) in the face causing disfiguration and difficulty with breathing.

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Symptoms of systemic scleroderma

Systemic sclerosis is capable of affecting the connective tissue in almost every part of the body. Because it changes everything from skin to the gastrointestinal tract, lungs, kidneys, heart and other internal organs. The hardening of tissue in the organs can cause them to work improperly which can lead to many complications, including death. Symptoms of systemic scleroderma are:

  • losing hair;
  • calcium deposits;
  • dilated blood vessels under the skin’s surface;
  • joint problems such as pain;
  • trouble breathing;
  • coughing;
  • diarrhea;
  • constipation;
  • difficulty swallowing;
  • heartburn;
  • abdominal bloating after meals.

A special form of the limited scleroderma is the CREST syndrome, named aptly because of its symptoms.

Calcinosis - deposits of calcium forming in the skin;

Raynaud Phenomenon - spasms of the blood vessels in fingers and toes;

Esophageal dysfunction - trouble swallowing due to damaged muscles in the esophagus;

Sclerodactyly - tightness of skin on the fingers and toes;

Telangiectasia - dilated blood vessels usually found on the face.


There is no known cure for systemic scleroderma, but there are ways to treat the symptoms. Treatment is essential to prevent further complications. General symptoms are treatable with medicine such as:

  • corticosteroids;
  • immunosuppressants;
  • anti-inflammatory medicine.

If you are suffering from any of the symptoms or if you suspect you might have any form of scleroderma, contact your physician as soon as possible. Timely treatment of symptoms can be crucial in saving your life.

Source: HealthLine, EMedicine, MedicineNet, Scleroderma

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This article is purely for informational purposes. Do not self-medicate, and in all cases consult a certified healthcare professional before using any information presented in the article. The editorial board does not guarantee any results and does not bear any responsibility for harm that may result from using the information stated in the article.