Forms of Scleroderma

Forms of Scleroderma
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According to the Mayo Clinic, scleroderma is a disease that is characterized by an accumulation of collagen in the body's tissues. The immune system plays a role in scleroderma by attacking the body and inappropriately producing inflammation that results in collagen overproduction. The International Scleroderma Network (ISN) reports scleroderma affects an estimated one in 1,000 Americans. There are multiple subtypes of scleroderma that are named based upon the parts of the body affected.

Diffuse Scleroderma

The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) describes how the systemic form of scleroderma can affect any part of the body, including the skin, internal organs and blood vessels. The ISN describes how systemic scleroderma is divided into subtypes, including diffuse scleroderma. Diffuse scleroderma is diagnosed when there is tightness of the skin above the wrists. The ISN also reports that diffuse scleroderma can occur without involving the skin and affects only the digestive system connective tissue or other internal organs.

Limited Scleroderma

Limited scleroderma, the second subtype of systemic scleroderma, was named because the diagnosis involves tight skin that is limited to the fingers, according to the ISN. However, "limited" seems to be somewhat of a misnomer, as this subtype of scleroderma may also involve fibrosis of the lung and esophageal problems and may affect the skin of the face and neck.

Systemic Sclerosis Sine Scleroderma

A third subtype of systemic scleroderma, described by NIAMS, is systemic sclerosis sine scleroderma. "Sine" means "without," meaning that this manifestation of sclerosis is "without" scleroderma and does not affect the skin. The deposits of collagen still affect the internal organs, but there is no overt skin thickening.

Morphea

According to the Mayo Clinic, a second major type of scleroderma is "localized," which means it affects only the skin and not the internal organs. Like systemic scleroderma, localized scleroderma is divided further into subtypes. Morphea is one subtype of localized scleroderma. The ISN reports that morphea usually begins as patches of yellow- or ivory-colored skin that is rigid and tight. The ISN and Mayo Clinic describe how the patches become hard to the touch and usually have a white or yellowish center with a pink or purple halo. According to the ISN, these skin plaques usually appear on the trunk but can affect any part of the skin.

Linear Scleroderma

Linear localized scleroderma, the second subtype of localized scleroderma, is more common in children, according to the Mayo Clinic, and appears as bands or streaks of thick skin on one or both arms. These bands may also appear on the forehead.

References

Article reviewed by Bridget Gregory Last updated on: May 4, 2011

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