Definition & Overview

Scleroderma is an autoimmune disease that is characterized by the hardening of the skin. The term is derived from two Greek words, Sclero (hard) and Derma (skin).

Scleroderma is non-contagious, non-cancerous, and non-infectious. Although many cases of the disease are mild, some are life-threatening. Some forms are progressive, meaning that a mild case can progress into a serious case, but there are non-progressive cases as well.

The exact cause of the disease is unknown. It does not have any cure as well. However, treatments are available to control the symptoms.

Most cases of scleroderma appear in men and women between the ages of 20 and 50, but there are also cases that involve children.

Scleroderma has different forms, but has two main categories: Localized and Systemic. Under localized scleroderma are morphea and linear scleroderma. Systemic scleroderma has two forms as well, Limited and Diffuse.

Localized scleroderma is generally mild and occurs only on a few areas of the skin and muscles. Rarely will the disease spread to other areas of the body. Morphea, on the other hand, is a form of localized scleroderma that is characterized by waxy patches with hardened skin underneath. Linear scleroderma, also another form of localized scleroderma, is described as streaks or lines of waxy patches with a deeper layer of hardened skin underneath.

While localized scleroderma only affects skin and muscles, Systemic Scleroderma can affect internal organs as well. In some cases, the joints, blood vessels, and muscles are also affected.

Limited scleroderma, a form of systemic scleroderma, is described as the thickening of the skin limited to certain areas, such as the hands, face, and fingers. The disease develops slowly, but there is a chance that it will affect internal organs.

Limited scleroderma is also referred to as CREST, which stands for the five common features: Calcinosis, Raynaud Phenomenon, Esophageal Dysfunction, Sclerodactyly, and Telangiectasia.

On the other hand, Diffuse scleroderma develops rapidly and affects more areas of the skin. Patients with this condition are at risk of internal organ hardening.

Cause of Condition

Scleroderma is a result of the overproduction of collagen, a fibrous protein that makes up the skin and connective tissues. However, the exact cause of the overproduction and accumulation of collagen is yet to be determined.

According to studies, some of the possible causes are certain genes and the environment, which activates the immune system, damaging the blood vessels and causing scarring and excess production of collagen.

Although both men and women are prone to the disease, it occurs more often in women than men. Some races are also more prone to the disease than others.

Key Symptoms

The symptoms of scleroderma will largely depend on the type and form. Localized scleroderma mainly affects the skin, but sometimes the muscles as well. Localized scleroderma will display symptoms, such as waxy patches of skin (Morphea), or waxy streaks or lines on the skin (Linear). Since Linear scleroderma affects deeper layers of skin, it can also affect the joints.

Systemic scleroderma has more symptoms than localized scleroderma because this type also affects the internal organs, such as the heart, kidneys, lungs, esophagus, and gastrointestinal tract. It also affects the muscles and joints, which can result in decreased motion.

If the esophagus is affected, the patient will experience heartburn. It can also cause scarring of the esophagus, which will result in swallowing difficulties and pain in the central part of the chest. The patient may also experience elevated blood pressure, which can lead to a renal crisis, such as kidney damage. The common symptoms are headaches and fatigue. However, it’s important to note that elevated blood pressure can also lead to a stroke.

If the lungs are affected, the patient will experience shortness of breath. It can also result in pulmonary hypertension, which is an elevated pressure in the arteries leading to the lungs. On the other hand, if the heart is affected with scleroderma, the condition can cause irregular heart rhythms, abnormal electrical activity, and inflammation around the heart.

If scleroderma affects the colon, the patient will experience frequent constipation, leg cramps, or diarrhea. If the condition continues, it is likely to lead to stool blockage.

Who to See & Types of Treatment Available

If you notice waxy patches or lines on your skin, it’s best to consult your family doctor. If your doctor suspects the presence of scleroderma, you’ll be referred to a specialist, such as a dermatologist or internist.

To confirm scleroderma, the specialist will perform a series of blood tests to detect certain antibodies, such as the Anti-Sci 70, which is usually present in Diffuse scleroderma. Other types of tests will also be performed to determine if the disease has affected any of your internal organs.

It’s important to understand that there is no available cure for scleroderma. Once you have the disease, you’ll have it for the rest of your life. However, the symptoms of the disease can be treated to prevent them from developing into a more serious condition. If the condition is mild, there is a chance that you won’t require any form of treatment.

The treatment will depend on which internal organ has been affected. If hypertension is present, you’ll be given medications such as ACE inhibitors to control your blood pressure.

If scleroderma results in itchiness of the skin, you’ll be given lotions and other topical ointments.

Blood clots are a common concern for patients with scleroderma. The clots can be dislodged in the arteries and find their way into the heart, lungs, or brain, which is a life threatening condition. To reduce the chances of this happening, you’ll be given medications that can prevent the formation of blood clots.

If scleroderma affects your facial skin, laser treatments can be performed. You should also avoid sun exposure to prevent the condition from worsening.

Finally, since scleroderma is a result of an overactive immune system, it is likely that your doctor will prescribe medication to suppress your immune system.

References:

  • Varga J. Systemic Sclerosis (Scleroderma). In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 275.
  • Varga J. Etiology and Pathogenesis of Scleroderma. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap 83.
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