Scleroderma: how to recognize this autoimmune disease?

Scleroderma

158 people out of a million suffer from scleroderma. An update on this autoimmune disease with a dermatologist.

Scleroderma

Photo Karolina Grabowska in Pexels

Scleroderma: what exactly is it?

Systemic scleroderma (SCS) is a disease that affects between 6,000 and 8,000 adults. It is a predominantly female pathology since 8 out of 10 patients are women. It appears mainly between 45 and 65 years old.

Systemic Scleroderma: what is it? Systemic scleroderma is an autoimmune disease, which means it is a disorder of the immune system.

“For reasons not yet fully understood, people with systemic scleroderma suffer from permanent inflammation that results in a high production of poor quality collagen and a deposit of connective tissue under the skin and in certain organs,” explains Dr. Isabelle Gallay, a dermatologist.

Watch out! Systemic scleroderma (which can affect several organs in the body, including the lungs, heart, and digestive system) should not be confused with localized scleroderma, which only affects the skin.

Scleroderma: what are the symptoms?

Localized scleroderma manifests itself through very hard, rounded and well-defined areas of skin, especially on the forehead and/or scalp.

This area of hard skin (called “indurated” in medical language) is surrounded by a reddish-violet inflammatory ring. The patient usually has only a few of these.

Systemic Scleroderma

On the other hand, it usually starts with a Raynaud’s phenomenon that usually appears 5 to 10 years before the other signs of the disease.

“As the skin thickens and hardens, it compresses the blood vessels, especially in the fingers,” explains Dr. Gallay. As a result, the fingers become white (with a visible demarcation line on the first phalanx) and become less sensitive to cold and moisture.

The main symptom of systemic scleroderma is the hardening and thickening of the skin, which is particularly noticeable on the hands, feet and face.

“Scleroderma restricts movement: the joints are less mobile, the fingers retract, walking and bending the knees is difficult . ” says the dermatologist.

Added to this are inflammatory pain (which can wake up at night and/or prevent sleep) and mechanical pain (which is intensified by movement). Tolerance to cold and heat decreases.

The disease also tends to alter facial features,” adds Dr. Gallay. For example, the nose becomes thinner, the circumference of the mouth becomes thinner and with more stripes, the lips are almost invisible, the fingers are very thin…

Finally, systemic scleroderma can lead to the appearance of calcinosis: “these are calcareous deposits that form nodules (like “balls”) under the skin, particularly in the fingers,” says the specialist.

Scleroderma: what are the possible complications?

Unlike localized scleroderma, systemic scleroderma is not limited to the skin: this autoimmune disease can affect different organs, with more or less severe complications:

  • The digestive system. “In most cases, the disease reaches the esophagus, which causes a postural gastroesophageal reflux, with acid reflux occurring more often when sitting or lying down,” Dr. Gallay says,
  • Intestines. When systemic scleroderma reaches the intestines, chronic transit disorders and/or malabsorption can occur – which can lead to significant dietary deficiencies!
  • The heart. Myocardial fibrosis (i.e., hardening and thickening of the heart muscle) can lead to potentially serious heart rhythm abnormalities and ventricular dysfunction.
  • Lungs. When systemic scleroderma reaches the lungs, it can develop into pulmonary fibrosis: a very serious complication that can be responsible for poor blood oxygenation.
  • Kidneys. Renal crisis due to scleroderma occurs in 10% to 20% of patients: it corresponds to a sudden narrowing of the small blood vessels that supply the kidneys and can lead to acute renal failure? potentially fatal.

Diagnosis and treatment: what treatment for scleroderma?

Systemic scleroderma is diagnosed by the dermatologist first with a complete clinical examination and then with a blood test to identify the inflammatory syndrome.

“Patients are followed by several health professionals to monitor disease progression, including cardiac, pulmonary and renal function tests (on the ventilator), electrocardiograms (at the cardiologist) and regular urinalysis,” adds Dr. Gallay.

Unfortunately, there is currently no cure for systemic scleroderma. To relieve the symptoms of the condition, dermatologists may prescribe pain medications (analgesics) and/or corticosteroids to calm inflammation.

“When calcinosis is present, local emollients are also prescribed, says the specialist. And if the disease is truly disabling, immunosuppressive treatment can be started.

And also… “Good sun protection is essential when suffering from scleroderma: it is a matter of protecting the skin and blood vessels, but also ‘good’ collagen,” says Dr. Gallay.

Doctors also recommend regular physical activity, ideally outdoors: walking, biking “At the same time, physical therapy and/or osteopathy sessions may be recommended. “

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