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Scleroderma - systemic sclerosis

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Scleroderma literally means "hard skin." Scleroderma is one of a group of arthritic conditions called connective tissue diseases.

The cause for scleroderma is unknown. It's more common in women than in men and more common in adults than in children. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. It's not considered contagious or cancerous.

Signs and symptoms

In addition to thickening and hardening of your skin, scleroderma can cause your skin to lose its elasticity and become shiny as it stretches across underlying bone. Other signs and symptoms may include:

  • Numbness, pain or color changes in your fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress (Raynaud's phenomenon)

  • Stiffness or pain in your joints and curling of your fingers

  • Digestive problems ranging from poor absorption of nutrients to delayed movement of food due to impaired muscular activity in the intestine

  • Sores over joints, such as your elbows and knuckles

  • Puffy hands and feet, particularly in the morning


Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein found in connective tissues, including your skin.

Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen. In addition to its effects on your skin, scleroderma affects tiny blood vessels and can affect almost every organ.

Doctors classify scleroderma into different subsets:

Diffuse cutaneous systemic sclerosis
This type affects the skin of your fingers, hands, arms, legs, face, neck and trunk. Internal organs such as your lungs, heart, gastrointestinal tract — including your esophagus — and kidneys may be affected. It can hinder the functions of your digestive system, create respiratory problems and cause kidney failure. Untreated, systemic scleroderma may be fatal within several years of onset.

Limited cutaneous systemic sclerosis
This type involves the skin of your fingers, lower arms and legs, face and neck. A variation is called CREST syndrome. CREST stands for these conditions:

  • Calcinosis — calcification in the skin

  • Raynaud's phenomenon

  • Esophageal dysfunction — such as reflux or difficulty in swallowing

  • Sclerodactyly — hardening of the skin of the fingers or toes

  • Telangiectasia — dilatation of tiny blood vessels, particularly of the skin

Localized scleroderma
This type affects mainly your skin and deep tissues below your skin. It includes the following subclassifications:

  • Morphea. In this form, oval-shaped thick patches appear on your skin — white in the middle, with a purple border. These patches are most likely to occur on your torso, but they can also appear on your arms, legs or forehead.

  • Linear scleroderma. This form results in bands or streaks of hardened skin on one or both of your arms or legs, or on your forehead.

Overlap syndrome
This is diffuse or limited systemic sclerosis with features of one or more of the other connective tissue diseases. Mixed connective tissue disease (MCTD) is an overlap syndrome with features of scleroderma, systemic lupus erythematosus, polymyositis and rheumatoid arthritis, and with the presence of an antibody produced by your immune system that is directed against cellular proteins in your body.

Undifferentiated connective tissue disease
This has features of systemic sclerosis, but there are no definite clinical or laboratory findings to make a definite diagnosis.

Sine scleroderma
Some doctors may describe one variation of scleroderma as sine scleroderma, which can be similar to either limited or diffuse scleroderma, the difference being that this form doesn't affect your skin. Sine is the Latin word for "without."

Scleroderma - systemic sclerosis > next > 1 > 2 > 3 > 4

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