Scleroderma, or systemic sclerosis, is an autoimmune connective tissue disorder, characterized by a progressive hardening of the skin. In some people, only the skin is affected, but in many people, scleroderma also harms structures beyond the skin — such as blood vessels, internal organs and the digestive tract. Scleroderma affects women more often than men and most commonly occurs between the ages of 30 and 50. While there is no cure, a variety of treatments can ease symptoms and improve quality of life.
Signs and symptoms vary, depending on which parts of your body are affected:
- Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted.
- Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud’s phenomenon, this condition also occurs in people who don’t have scleroderma.
- Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren’t moving food properly through the intestines.
- Heart, lungs or kidneys. Rarely, scleroderma can affect the function of the heart, lungs or kidneys. These problems can become life-threatening.
Current treatments include medications that modulate the immune system, chemotherapy drugs, vasodilators and ACE-inhibitors. Presently, most treatments act to slow the progression of the disease and limit damage rather than truly arresting the disease.