Scleroderma is a term used to refer to group of autoimmune disorders, which affects the connective tissue and skin. These diseases cause hardening and tightening of skin and other parts of the connective tissue or fibers providing framework and support for the body. Scleroderma may only affect the skin in some people but in others, it causes damages to other parts of the body besides the skin such as the internal organs, blood vessels, and digestive tract.
This condition is prevalent in women than in men and it manifests in ages of around 30 to 50 years. Scleroderma conditions are broadly grouped into two types, namely localized and generalized scleroderma. The localized scleroderma affects certain areas of the body. Morphea is a localized type of the condition, which involves oval shaped patches on the skin, which are hard. These patches start as purple or red spots, which then form a whitish center.
Linear localized scleroderma forms lines and streaks that have a thick skin and mainly affects the face, legs and arms. Of the generalized scleroderma conditions, there is limited and diffuse scleroderma. The limited scleroderma manifests gradually and affects the skin on face, feet and hands. It can advance to cause damage on organs like the lungs, intestines, and oesophagus. Diffuse scleroderma occurs more rapidly during its onset and affects the skin on trunk, upper arms and thighs. It can also affect internal organs like lungs, heart, and kidneys.
What are the causes?
It is not known what exactly causes scleroderma but scientists believe that the condition occurs due to an autoimmune response where the immune system begins to cause inflammation of skin and organs. The condition triggers the body to synthesize more collagen, a protein that gives the skin its structure. The result is a thickened and tightened skin as well as scarring internal organs.
Collagen forms the connective tissue fibrous network, which gives the structure of the organs including the skin, blood vessels, lungs, heart, and other organs. When the blood vessels are affected by abnormal spasm and thickening, they can result to damage of tissue and increased blood pressure within the pulmonary and systemic arteries.
Symptoms can vary depending on the areas, which are affected. If the skin is affected, as happens to nearly all people with the condition, it hardens and thickens causing patches of oval shapes or straight lines. The skin also appears shiny since it is tight and has limited movement. An early sign of scleroderma is Raynaud’s phenomenon, which affects the fingers and toes causing abnormal reaction to cold temperatures and emotional distress. The toes may be numb, change their color, and become painful. The Raynaud’s phenomenon leads to red, blue, and white discoloration of the fingers and at times, it can cause ulceration.
Scleroderma can also affect the digestive system, whereby it causes an increase in acid that damages the esophagus near the stomach. There may be problems in absorption of nutrients through the intestinal wall because the intestine muscles may not be able to move food smoothly down the intestines. When the scleroderma affects organs like the lungs, heart, and kidneys, it can become life threatening though this happens rarely.
Scleroderma affects many parts of the body something that can make it difficult to diagnose. Doctors may check if there are elevated levels of antibodies produced from the immune reaction. At times, scleroderma that affects the skin can subside and go away on its own. This may occur between three to five years since its onset. However, when internal organs are affected, they usually worsen over time.
There is no any drug that has been specifically developed to treat scleroderma and therefore most of the medications and therapies are aimed at treating its symptoms. In essence, there is no drug that can stop the overproduction of collagen, which causes the symptoms of scleroderma on the skin, blood vessels, and organs. Medications like blood vessel dilators may be used to counter increased blood pressure and prevent problems in organs like kidneys and lungs.
Medications to suppress the immune system may be used to reduce the reaction that causes the production of collagen. When ulceration occurs in the finger tips because of the Raynaud’s phenomenon, antibiotics may be used to prevent infections. Pain may be relieved with use of pain relievers. To manage the pain, nonsteroidal anti-inflammatory drugs- NSAIDs may be used to treat the inflammation. These include ibuprofen and aspirin.
Heartburn medication is recommended if the acid influx in the oesophagus is causing troubles. Physical and occupational therapy can be applied to manage pain, improve mobility, and strengthen the body. As a last resort, surgery may be used to treat complications of scleroderma such as damages of the lungs and blood vessels. Amputations may be done on fingers when severe Raynaud’s phenomenon causes ulcers that develop into gangrene. Organ transplant of the lungs may be performed if pulmonary hypertension damages of the lungs.