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Scleroderma

A chronic disorder marked by hardening and thickening of the skin.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Scleroderma

Derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin, scleroderma literally means "hard skin." Although it is often referred to as if it were a single disease, scleroderma is really a symptom of a group of diseases that involve the abnormal growth of connective tissue, which supports the skin and internal organs. It is sometimes used, therefore, as an umbrella term for these disorders. In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. In other forms, however, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Scleroderma is called both a rheumatic (roo-MA-tik) disease and a connective tissue disease. The term rheumatic disease refers to a group of conditions characterized by inflammation or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage....Read more about Scleroderma NIH - National Institute of Arthritis and Musculoskeletal and Skin Diseases

What works? Research summarized

Evidence reviews

Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis

Scleroderma is a connective tissue disease causing fibrosis and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart.

Prazosin for Raynaud's phenomenon in progressive systemic sclerosis

Scleroderma is a connective tissue disease causing fibrosis and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart. Most people with scleroderma also have raynaud's phenomenon (RP). RP is defined as vasospasm of arteries or arterioles causing pallor and at least one other colour change upon reperfusion such as cyanosis or redness. Primary RP occurs in the absence of causes such as connective tissue disease. Secondary RP occurs in people with underlying diseases that affect blood vessels especially scleroderma and lupus. The RP that occurs in scleroderma is often more severe in that there is not only vasospasm but also a fixed blood vessel deficit with intimal proliferation and therefore narrowing of the blood vessels. Raynaud's phenomenon may also be accompanied by digital ulcers which are possibly secondary to ischemia.

Ketanserin is a drug that has been studied in the treatment of Raynaud's phenomenon and associated conditions. It is not widely used however.

Raynaud's phenomenon is a disease that causes decreased blood flow and circulation to the extremeties. Symptoms include discolouration, pain, and in some severe cases ulceration of the hands and feet. It is most often triggered by cold, stress, and emotional discomfort. Primary Raynaud's phenomenon has no underlying disease associated with it. Secondary Raynaud's phenomenon is most often associated with scleroderma, but may also be related to systemic lupus erythematosus, mixed connective tissue disease, Sjorgen's syndrome, dermatomyositis, or rheumatoid arthritis. Scleroderma is a connective tissue disease causing hardening and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart.

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Summaries for consumers

Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis

Scleroderma is a connective tissue disease causing fibrosis and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart.

Prazosin for Raynaud's phenomenon in progressive systemic sclerosis

Scleroderma is a connective tissue disease causing fibrosis and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart. Most people with scleroderma also have raynaud's phenomenon (RP). RP is defined as vasospasm of arteries or arterioles causing pallor and at least one other colour change upon reperfusion such as cyanosis or redness. Primary RP occurs in the absence of causes such as connective tissue disease. Secondary RP occurs in people with underlying diseases that affect blood vessels especially scleroderma and lupus. The RP that occurs in scleroderma is often more severe in that there is not only vasospasm but also a fixed blood vessel deficit with intimal proliferation and therefore narrowing of the blood vessels. Raynaud's phenomenon may also be accompanied by digital ulcers which are possibly secondary to ischemia.

Ketanserin is a drug that has been studied in the treatment of Raynaud's phenomenon and associated conditions. It is not widely used however.

Raynaud's phenomenon is a disease that causes decreased blood flow and circulation to the extremeties. Symptoms include discolouration, pain, and in some severe cases ulceration of the hands and feet. It is most often triggered by cold, stress, and emotional discomfort. Primary Raynaud's phenomenon has no underlying disease associated with it. Secondary Raynaud's phenomenon is most often associated with scleroderma, but may also be related to systemic lupus erythematosus, mixed connective tissue disease, Sjorgen's syndrome, dermatomyositis, or rheumatoid arthritis. Scleroderma is a connective tissue disease causing hardening and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart.

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Terms to know

Connective Tissue
Supporting tissue that surrounds other tissues and organs. Specialized connective tissue includes bone, cartilage, blood, and fat.
Inflammation
Redness, swelling, pain, and/or a feeling of heat in an area of the body. This is a protective reaction to injury, disease, or irritation of the tissues.
Raynaud's
Reduced blood flow in response to cold or emotional stress, causing discoloration of the fingers, toes, and occasionally other areas. May also cause nails to become brittle.
Rheumatologist
Doctors who diagnose and treat diseases of the bones, joints, muscles, and tendons, including arthritis and collagen diseases.
Skin
The outer covering of the body that protects it from the environment.

More about Scleroderma

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Other terms to know: See all 5
Connective Tissue, Inflammation, Raynaud's

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