What is Scleroderma?

Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.

The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease.

Scleroderma involves an overproduction of collagen and presents itself in two major forms:

  • Localized Scleroderma is characterized by inflammation and thickening of the skin from excessive collagen deposition. Relatively mild, only a few areas of the body are affected — usually the skin or muscles. Localized scleroderma rarely becomes systemic, Internal organs are typically not affected.

 

  • Systemic Scleroderma or Systemic sclerosis (SSc) is a multi-system autoimmune disease that results in fibrosis (development of scar tissue), vascular damage, and abnormal connective tissue growth in many parts of the body including muscles and joints. These abnormalities lead to the breakdown of the skin, subcutaneous tissue, muscles, and internal organs such as the heart, lungs, kidneys, and intestinal tract.

 

What are the symptoms?

This disease can manifest in different ways. Among the early signs of scleroderma is Raynaud’s phenomenon (pronounced RAY-KNOWDS), which is identified by fingers that become very sensitive to cold, causing the fingers to change colors. Hardiness of the skin, shortness of breath, difficulty swallowing, edema are among the symptoms that a patient could present. 

 

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What causes scleroderma?

At the moment there is no known cause of scleroderma and no way of preventing this disease. It is more common in females than males, yet it affects all genders, all ages and all racial groups. The peak of the disease onset occurs in the fourth and fifth decades of life. Familial scleroderma is rare, and there is no evidence of genetic inheritance.

How does scleroderma affect the patients who are living with this disease?

The symptoms and effects of this disease vary greatly from patient to patient. When the skin is affected, the symptoms are visible, but, when only internal organs are affected, they are unseen. For some, scleroderma can be life-threatening due to the severity of organ involvement, and, for others, their symptoms can be managed with the right treatment.

 

The effects of scleroderma also go beyond just the physical. Many scleroderma patients find that the emotional toll this disease takes can be equally as debilitating.

 

There is currently no cure for the hallmark symptom of scleroderma — skin thickening and hardening. In fact, no cure exists for the autoimmune disease at all, but different therapies are available to treat various conditions associated with scleroderma. Known as palliative therapies, these treatments help manage the disease’s wide range of conditions and their related symptoms, and to maintain a patient’s quality of life.