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Summary Article: Scleroderma from Encyclopedia of Global Health

Scleroderma, which is literally defined as “hard skin,” is an uncommon, chronic condition that produces thickening of the skin through an atypical growth of connective tissue. In more extensive and progressive cases, blood vessels and internal organs such as the lungs, esophagus, or intestines, may also be affected. Affected skin may appear erythematous (red) and firm to the touch. In addition, painful ulcers on the fingers or toes, joint pain/stiffness, and hair loss in affected areas may be seen. Important factors that help determine the severity of scleroderma include the total area affected by the disease, as well as the degree of involvement of the blood vessels and internal organs. While scleroderma can affect any age, race, or gender, occurrence within demographics may be seen. For example, localized scleroderma is more often found in people of European descent, whereas generalized scleroderma is more common in people of African-American descent. Generalized scleroderma is more likely to affect female adults (usually between the ages of 30–50). In the United States, approximately 300,000 individuals are affected by 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.

DIAGNOSIS

There are two important factors in discerning the diagnosis of scleroderma: clinical presentation and laboratory analysis. In addition to the hardening of the skin, clinical signs may be apparent. For example, a nonspecific sign that may be observed is Raynaud’s phenomenon (RP). RP is described as a change in skin color in the digits (fingers and toes) upon exposure to cold temperature, due to the contraction of blood vessels (ischemia). The fingertips often turn pale or white in cold temperature, and blue when lacking oxygen, a condition called cyanosis. If this condition persists, the lack of oxygen to these areas can elicit pain and ulcer formation. Additional signs of scleroderma may be calcinosis cutis (painful calcium deposition under the skin) or digestive impairment. Laboratory analysis is a vital component in the diagnosis which includes a biopsy of the involved skin and serologies for relevant antibodies, such as anticentromere or antitopoisomerase-1.

THERAPY

There is no known cure for any of the forms of scleroderma. However, depending on the severity of the disease in each individual case, treatments are available to manage the symptoms of the disease, such as antiinflammatory agents (such as celecoxib, prednisone, and the antibiotic minocycline) for joint pain or inflammation.

Steroid-sparing immunosuppressants (such as cyclosporine or azathioprine) have also been used effectively to moderate the disease. Physicians may also recommend certain lifestyle changes, such as smoking cessation and changes in eating habits. In addition, physical therapy may be needed to maintain functionality, especially when the limbs are involved.

    SEE ALSO:
  • Immunosuppresion; Skin Diseases (General).

BIBLIOGRAPHY
  • Mayo Clinic, “Scleroderma,” Mayo Clinic, www.mayoclinic.com/health/scleroderma/DS00362 (cited July 2006).
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases, “Handout on Health: Scleroderma,” www.niams.nih.gov/hi/topics/scleroderma/scleroderma.htm (cited July 2006).
  • Scleroderma Research Foundation, “Awareness and Outreach,” www.srfcure.org/srf/awareness.htm (cited July, 2006).
  • John Varga, “Overview of the Clinical Manifestations of Systemic Sclerosis (Scleroderma) in Adults,” www.uptodate.com (cited July 2006).
  • Shalu S. Patel

    Rajiv I. Nijhawan
    University of Michigan
    Copyright © 2008 by SAGE Publications, Inc.

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