Overview

Scleroderma (sklair-oh-DUR-muh) is a group of rare diseases, including skin and connective tissue hardening and tightening.

Scleroderma affects women more often than men and occurs most often between 30 and 50 years of age. While scleroderma is not healed, a number of treatments can reduce the symptoms and improve quality of life.

There are a number of different kinds of scleroderma. Scleroderma affects only the skin, in some cases. But scleroderma also affects tissues outside the skin in many cases, such as blood vessels, internal organs and the digestive tract (systemic scleroderma). Symptoms and signs vary, depending on what type of scleroderma you have.

Symptoms

The signs and symptoms of scleroderma differ depends to which parts of the body are affected:

• Skin: Nearly all those with scleroderma undergo a hardening and tightening of skin patches. These patches can be formed like ovals or straight lines, or they can cover large trunk and limb areas. The number, position, and size of the patches vary by scleroderma type. The skin may appear shiny because it’s so tight, and the affected area may be limited in movement.
• Fingers or Toes: Raynaud’s disease is one of the earliest signs of systemic scleroderma which causes the small blood vessels in your fingers and toes to contract in response to cold temperatures or emotional distress. Your fingers or toes can turn blue when this happens, or feel painful or numb. Raynaud’s disease also occurs in those without scleroderma.
• Digestive System: Depending on which part of the digestive tract is affected, scleroderma can cause a variety of digestive symptoms. When it affects the oesophagus you may have heartburn or trouble swallowing. You can get cramps, bloating, diarrhea or constipation if the intestines are affected. Some people with scleroderma can also have trouble consuming nutrients if their intestinal muscles don’t pass food through the intestines properly.
• Heart, Lungs or Kidneys: Scleroderma may affect the function of the heart, lungs or kidneys to a varying degree. Such conditions can become life-threatening if left untreated.

Causes

Scleroderma is the result of an overproduction and collagen accumulation in body tissues. Collagen is a type of fibrous protein that makes up the connective tissues of your body including your skin.

Doctors don’t know exactly what causes the irregular development of collagen to start, but the body’s immune system seems to play a role. Most likely, a combination of factors causes scleroderma, including issues with the immune system, genetics and environmental triggers.

Risk factors

Anyone can get scleroderma but in women, it occurs much more frequently than in men. The chance of developing scleroderma seems to be affected by several combined factors:

• Genetics: People with certain variations of the genes appear to be more likely to develop scleroderma. This can explain why a limited number of cases of scleroderma tend to be occurring in families and why certain forms of scleroderma are more common in certain ethnic groups. Choctaw Native Americans, for instance, are more likely to acquire the form of scleroderma that affects the internal organs.
• Environmental Triggers: Research suggests that exposure to certain viruses, medicines, or drugs can cause scleroderma symptoms in some people. Repeated exposure to such toxic substances or chemicals — such as at work — can also raise the risk of scleroderma.
• Immune System Problems: It is suspected that scleroderma is an autoimmune condition. This means it occurs in part because the body’s immune system begins destroying the connective tissues. An individual with scleroderma also has symptoms of another autoimmune disorder in 15 to 20 per cent of cases, such as rheumatoid arthritis, lupus, or Sjogren’s syndrome.

Complications

Complications of scleroderma vary from mild to extreme, and can affect:

Fingertips: The variety of Raynaud’s disease that occurs with systemic scleroderma can be so serious that the limited blood flow permanently affects the on fingertips the tissue, creating pits or sores of the skin. In some cases, the fingertip tissue may die and require amputation.

Lungs: Lung tissue scarring can result in decreased lung function, which can affect your breathing ability and exercise tolerance. High blood pressure in the arteries to your lungs can also build.

Kidneys: You can develop elevated blood pressure and an increased level of protein in your urine when scleroderma affects your kidneys. More severe kidney complications effects may involve renal crisis, causing a sudden rise in blood pressure and rapid failure of the kidneys.

Heart: Scarring of heart tissue increases your risk of abnormal heartbeats and congestive heart failure and can cause inflammation of your heart’s membrane sac. Scleroderma can also increase the pressure on your heart’s right side and cause it to wear out.

Teeth: Severe stretching of the facial skin will make the mouth small and narrow, which can make it harder to brush your teeth or even get them cleaned professionally. People who have scleroderma also do not produce regular quantities of saliva, and there is an even greater chance of dental decay.

Digestive System: Scleroderma-related digestive problems can cause heartburn and trouble swallowing. Also, it may cause cramp spells, bloating, constipation, or diarrhea.

Sexual Function: Men with scleroderma can get erectile dysfunction. Scleroderma may also affect women’s sexual function by reducing sexual lubrication and restricting vaginal opening.