Wednesday, March 3, 2010

Learning about Scleroderma

Scleroderma is an autoimmune disease of the connective tissue. Autoimmune diseases are illnesses which occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body. This leads to thickness and firmness of involved areas. Scleroderma, when it's diffuse or widespread over the body, is also referred to as systemic sclerosis.

Patients can have variations of CREST, for example, CRST, REST, ST, etc. Patients can also have "overlap" illness with features of both CREST and the diffuse form of scleroderma. Some patients have overlaps of scleroderma and other connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, andpolymyositis. When features of scleroderma are present along with features of polymyositis and systemic lupus erythematosus, the condition is referred to as mixed connective tissue disease (MCTD).

Sclerodactyly refers to the localized thickening and tightness of the skin of the fingers or toes. This can give them a "shiny" and slightly puffy appearance. The tightness can cause severe limitation of motion of the fingers and toes. These skin changes generally progress much slower that those of patients with the diffuse form of scleroderma.

The symptoms of scleroderma depend on the type of scleroderma present and the extent of external and internal involvement in the individual affected. Because scleroderma can affect the skin, esophagus, blood vessels, kidneys, lungs, blood pressure and bowels, the symptoms it causes can involve many areas of the body.

Scleroderma affects the skin to cause local or widespread signs of inflammation (redness, swelling, tenderness, itching, and pain) that can lead to skin tightness or hardening. These skin changes can be widespread, but it's most common for them to affect the fingers, feet, face, and neck. This can lead to decreased range of motion of the fingers, toes, and jaw. Tiny areas of calcification (calcinosis), while not common, can sometimes be noticed as hard nodules at the tips of the elbows or in the fingers.

Raynaud's phenomenon (RP) is a condition resulting in a particular series of discolorations of the fingers and/or the toes after exposure to changes in temperature (cold or hot) or emotional events. Skin discoloration occurs because an abnormal spasm of the blood vessels causes a diminished blood supply to the local tissues. Initially, the digit(s) involved turn white because of the diminished blood supply. The digit(s) then turn blue because of prolonged lack of oxygen. Finally, the blood vessels reopen, causing a local "flushing" phenomenon, which turns the digit(s) red. This three-phase color sequence (white to blue to red), most often upon exposure to cold temperature, is characteristic of RP.

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 and can cause serious heart and lung disorders.

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 throat. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.

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