Limited scleroderma

DEFINITION

Limited scleroderma, or CREST syndrome, is one subtype of scleroderma — a condition that literally means “hardened skin.”

The skin changes associated with limited scleroderma typically occur only in the lower arms and legs and sometimes the face and throat. Limited scleroderma can also affect your digestive tract.

The problems caused by limited scleroderma may be minor. Sometimes, however, the disease affects the lungs or heart, with potentially serious results. Limited scleroderma has no known cure, and treatments focus on managing symptoms and preventing serious complications.

CAUSES

Limited scleroderma is believed to be an autoimmune disorder — one in which your immune system turns against your own body. In limited scleroderma, the immune system appears 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, impairing their function.

SYMPTOMS

While some varieties of scleroderma occur rapidly, signs and symptoms of limited scleroderma usually develop gradually. They include: 

• Tight, hardened skin. In limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and neck. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.
• Raynaud’s phenomenon. This condition occurs when small blood vessels in your fingers and toes go into spasms in response to cold or emotional stress, blocking the flow of blood. In most people, the skin turns white before becoming blue, cold and numb. When circulation improves, the skin usually reddens and may throb or tingle. Raynaud’s phenomenon is often one of the earliest signs of limited scleroderma, but many people have only Raynaud’s and never develop scleroderma.
• Red spots or lines on skin. These small red spots or lines (telangiectasias) are caused by the swelling of tiny blood vessels near the skin’s surface. They are not painful and occur primarily on the hands and face.
• Bumps under the skin. Limited scleroderma may cause tiny calcium deposits (calcinosis) to develop under your skin, mainly on your elbows, knees and fingers. You can see and feel these deposits, which sometimes may be tender or become infected.
• Swallowing difficulties. People with limited scleroderma commonly experience problems with their esophagus — the tube that connects the mouth and stomach. Poor functioning of the muscles in the upper and lower esophagus can make swallowing difficult and allow stomach acids to back up into the esophagus, leading to heartburn, inflammation and scarring of esophageal tissues.