Scleroderma

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Types

There are two basic types of scleroderma: localized and generalized. In most cases, localized scleroderma is a more mild form of the disease. It is characterized by thickening or hardening of the skin on the arms, legs, and face. Localized scleroderma usually does not affect the trunk (chest, back, and abdomen).

Two forms of localized scleroderma are morphea, which causes hard, oval-shaped patches on the skin, and linear, which causes lines or streaks of hardened skin on the arms, legs, and forehead.

Localized scleroderma features CREST syndrome, which involves the following:

• Calcinosis (abnormal calcium deposits in the skin)
• Raynaud's syndrome (reduced blood flow to the extremities causing numbness, tingling, and burning; also called Raynaud’s phenomenon)
• Esophageal dysfunction (e.g., reflux)
• Sclerodactyly (hardening of the skin of the fingers and toes)
• Telangiectasia (lesion caused by dilation of a group of blood vessels)

Generalized scleroderma may affect many parts of the body. It often involves thickening of the skin of the arms, legs, and trunk. There are two basic types of generalized scleroderma: limited and diffuse.

Limited scleroderma usually develops gradually over time. It affects the skin first and then affects organs (e.g., gastrointestinal tract, heart, lungs, kidneys). In limited scleroderma, early symptoms (e.g., Raynaud's syndrome) often occur years before development of the disease. Diffuse scleroderma develops rapidly and causes thickening of the skin throughout the body. This type often affects internal organs.

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