Scleroderma is a connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs. It is a type of autoimmune disorder, a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue.
The cause of scleroderma is unknown. People with this condition have a buildup of a substance called collagen in the skin and other organs. This buildup leads to the symptoms of the disease.
The disease usually affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.
Some types of scleroderma affect only the skin, while others affect the whole body.
Localized scleroderma usually affects only the skin on the hands and face. It develops slowly, and rarely, if ever, spreads throughout the body or causes serious complications.
Systemic scleroderma, or sclerosis, may affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types of systemic scleroderma: Limited disease (CREST syndrome) and diffuse disease.
Skin symptoms of scleroderma may include:
Fingers or toes that turn blue or white in response to hot and cold temperatures (See: Raynaud's phenomenon)
Skin that is abnormally dark or light
Skin thickening, stiffness, and tightness of fingers, hands, and forearm
Small white lumps beneath the skin, sometimes oozing a white substance that looks like toothpaste
Sores (ulcers) on the fingertips or toes
Tight and mask-like skin on the face
Bone and muscle symptoms may include:
Numbness and pain in the feet
Pain, stiffness, and swelling of fingers and joints
Breathing problems may result from scarring in the lungs and can include:
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Ariel D. Teitel, MD, MBA, Clinical Associate Professor of Medicine, NYU Langone Medical Center. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.