Scleroderma

Localized Scleroderma

Localized scleroderma is designated, when only the skin is affected. The two types of localized scleroderma are called morphea and linear. The morphea-type scleroderma is characterized by thick, oval-shaped plaques with a bluish-purple rim. Ablation or reduction of the hair follicle with resultant reduced hair growth and diminished sweating is characteristic. This type of localized scleroderma has been found more commonly in people aged 20–40. Linear scleroderma, on the other hand, may be seen as bands of hardened skin. In the case that it occurs on the head, it is termed en coup de sabre. Notably, this subtype is more prevalent in children and teenagers.

Generalized Scleroderma

Generalized scleroderma is another subtype of scleroderma in which multiple parts of the body are affected. Generalized scleroderma also has two different types: diffuse (DcSSc) and limited (LcSSc). Both forms of systemic sclerosis are known to affect internal organs and blood vessels as well as the skin. Almost 90 percent of these patients have gastrointestinal (GI) involvement, even though half of them might not even realize it. Common GI symptoms include difficulty swallowing, cough after swallowing, feelings of choking, heartburn, constipation or diarrhea, and these mainly stem from chronic gastroesophageal reflux. DcSSc is known to be the most severe type and thus the most life threatening, because of its rapid onset and extensive influence on the skin and internal organs. LcSSc, however, has a better prognosis, as the affects on the skin and internal organs are less severe in extent and character. The onset tends to be slower onset.

Causes

Scleroderma occurs primarily from the overproduction of a connective tissue protein called collagen. While the exact reason has yet to be determined, it is known that the immune system plays a role in the excess production of the collagen by activated fibroblasts (cells responsible for collagen production). The increase in collagen leads to thick bands forming (fibrosis) in the skin and around internal organs. Because these fibrotic bands are nonpliable, they may restrict the function of the involved tissue. Some researchers theorize that hormones such as estrogen play a significant role because this condition is much more common in women between 30 and 55 years of age.

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