overview
Limited scleroderma, also known as CREST syndrome, is a subtype of scleroderma – a condition whose name means “hardened skin”.
Skin changes associated with limited scleroderma usually only appear in the forearms and legs, under the elbows and knees, and sometimes affect the face and neck. Impaired scleroderma can also affect your digestive tract, heart, lungs, or kidneys.
The problems caused by limited scleroderma can be minor. However, sometimes the disease affects the lungs or heart, with potentially serious consequences. Limited scleroderma has no known cure. Treatments focus on managing symptoms, preventing serious complications, and improving quality of life.
symptoms
- Fingers affected by limited scleroderma
- Impaired Scleroderma Pop-up Dialog Open Hands Affected by Raynaud’s Disease
- Raynaud’s Disease Open pop-up dialog
- Telangiectasia Open the pop-up dialog box
- While some types of scleroderma come on quickly, the signs and symptoms of limited scleroderma usually develop gradually. They include:
Tight and hardened skin. With limited scleroderma, skin changes usually affect only the forearms and legs, including the fingers and toes, and sometimes the face and neck. The skin can appear shiny when stretched over the underlying bone. It can be difficult to bend your fingers or open your mouth.
The reasons
The cause is unknown, but limited scleroderma is believed to be an autoimmune disease in which your immune system turns against your body. The immune system seems to stimulate the production of too much collagen, a key component of connective tissue. This overproduction of collagen builds up in the skin and internal organs so they don’t function normally.
Risk factors
- Your Gender Women are much more likely to develop impaired scleroderma than men.
- Age. Impaired scleroderma is more common between the ages of 30 and 50.
- Run. In the United States, limited scleroderma tends to be more severe in blacks and Native Americans than in whites.
- Genetic factors. If someone in your family has an autoimmune disease – such as lupus, rheumatoid arthritis, or Hashimoto’s disease – there is an increased risk of developing limited scleroderma.
- Exposure to toxins. Certain toxic substances such as polyvinyl chloride, benzene, silica, and trichlorethylene can cause scleroderma in people with a genetic predisposition to the disease.
Complications
The visible signs of limited scleroderma – tight, thick skin on your fingers, hands, and face – can change the way you look. make everyday tasks like opening a glass or shaving difficult; and influence your language. However, the most serious complications often occur under the skin.
Gastrointestinal problems. Changes in the way the muscles of the esophagus work can lead to difficulty swallowing and chronic heartburn. If impaired scleroderma affects your bowels, it can lead to constipation, diarrhea, gas after meals, accidental weight loss, and malnutrition.
Ulcers on fingers and toes. Severe Raynaud’s phenomena can obstruct the flow of blood to your fingers and toes and cause ulcers that can be difficult to heal. In addition, abnormal or narrowed blood vessels associated with severe Raynaud’s phenomena can burn the fingers or toes that may require amputation.
Lung damage. Limited scleroderma can cause a variety of lung problems. In some cases, excess collagen builds up in the tissue between the air sacs of the lungs, making the lung tissue stiffer and less functional.