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Scleroderma

(Systemic Sclerosis)

Pronounced: Sclare-oh-DER-mah

Definition

Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are 3 major forms of the disease:

  • Localized scleroderma—Usually affects only the skin in isolated parts of the body. This form is less serious.
    • Morphea—local patches of scleroderma
    • Linear scleroderma—single line or band of thickened or abnormally colored skin.
  • Systemic scleroderma (sclerosis)—Affects widespread areas of skin and/or internal organs, most often the lungs. Also involves Raynaud phenomenon. Certain categories of this form of scleroderma are more serious and can be fatal.
    • Limited
      • A gradually progressing form of scleroderma that initially causes skin thickening, most commonly on the hands, forearms, feet, and lower legs
      • Usually progresses to affect the internal organs.
    • Diffuse
      • A more quickly progressing form of scleroderma that causes the skin to thicken throughout the body.
      • It may also affect the internal organs.
    • Sclerosis sine scleroderma—very rare form of scleroderma in which there are no skin manifestations, but the internal organs are affected.
  • Overlap syndrome—May involve features of scleroderma and features of other autoimmune syndromes
  • Undifferentiated scleroderma—Involves Raynaud phenomenon and a few aditional features of systemic sclerosis.

Causes    TOP

Overproduction of collagen and other connective tissue proteins are the main features of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production.

Risk Factors    TOP

Scleroderma is more common in women. The morphea type of scleroderma usually affects people 20-40 years old. Linear scleroderma often occurs in children. Systemic scleroderma is more likely to occur in people aged 30-50 years old.

Other factors that may increase your risk of developing scleroderma include:

  • Family members with systemic sclerosis or other autoimmune disorders, such as systemic lupus erythematosus
  • Occupational chemical exposure, such as silica, ketones, or welding fumes

Symptoms    TOP

Localized Scleroderma

Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:

  • Hard patches on the skin, most often on the face or trunk (morphea)
  • Lines of plaques or thickened skin.
  • Thickening can extend to underlying muscles and bones (linear scleroderma or linear morphea)
  • Muscle and joint aches, and tiredness may occur with morphea

Systemic Scleroderma

This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with Raynaud's phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. The fingers turn red after resolution of the symptoms. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.

Raynaud's Phenomenon Symptom

Low blood flwo to fingers, vasoconstriction
Copyright © Nucleus Medical Media, Inc.

Diffuse scleroderma comes on more suddenly and can progress to involve not only the skin but the internal organs. Other symptoms of diffuse scleroderma may include:

  • Diffuse thickening and hardening of the skin
  • Joint and muscular pain, stiffness, and swelling
  • Problems with breathing, swallowing, and digesting food due to thickening and hardening of lung, esophagus, bowel tissues
  • Inflammation and thickening of large and small blood vessels

Complications of diffuse scleroderma can affect virtually every system of the body. Other common complications include:

Diagnosis    TOP

You will be asked about your symptoms and medical history. A physical exam will be done. Diagnosis is based on changes in the skin.

Tests may include:

  • Blood tests—results may be typical of scleroderma, but do not prove it
  • Biopsies of skin and other tissues

Imaging tests take pictures of internal body structures. These may include:

In addition, an esophagus motility study may be done

Treatment    TOP

There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms in various systems.

Joints and Muscles

  • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and naproxen
  • Corticosteroids

Esophagus and Digestive Tract

  • Medications to limit acid production in the stomach
  • Small, frequent meals
  • Sleeping with your head elevated to avoid acid reflux

Skin and Internal Organs    TOP

  • Corticosteroids
  • Immunosuppressants
  • Prostanoids—drugs that control the immune system
  • Phototherapy

Kidney Problems and/or High Blood Pressure    TOP

  • ACE inhibitors, or other antihypertensive drugs
  • Antineoplastic agents
  • Vasodilators

Raynaud's Phenomenon    TOP

  • Calcium channel blockers
  • Proper shelter and clothing to avoid cold
  • Prostanoids—drugs that control the immune system
  • If you smoke, talk to your doctor about ways to quit.

Restricted Mobility    TOP

Physical therapy and exercise are important to maintain circulation, joint flexibility, and muscle strength.

Prevention    TOP

There are no current guidelines to prevent scleroderma.

RESOURCES:

Scleroderma Foundation
http://www.scleroderma.org
Scleroderma Research Foundation
http://www.srfcure.org

CANADIAN RESOURCES:

Arthritis Society
http://www.arthritis.ca
Scleroderma Society of Canada
http://www.scleroderma.ca

References:

Kreuter A, Hyun J, et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440-447.
Localized scleroderma. EBSCO DynaMed website. Available at: http://www.dynamed.... Updated June 4, 2013. Accessed November 29, 2016.
Mathai SC, Girgis RE, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007;29:469-475.
Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity. 2005;38:219-223.
Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at:
...(Click grey area to select URL)
Updated August 2016. Accessed November 29, 2016.
Systemic sclerosis. EBSCO DynaMed website. Available at: http://www.dynamed.com/topics/dmp~AN~T116347/Systemic-sclerosis. Updated June 9, 2016. Accessed November 29, 2016.
What is scleroderma? Scleroderma Foundation website. Available at:
...(Click grey area to select URL)
Accessed November 29, 2016.
11/9/2015 DynaMed's Systematic Literature Surveillance
...(Click grey area to select URL)
Kuo CF, Grainge MJ, Valdes AM, et al. Familial aggregation of systemic lupus erythematosus and coaggregation of autoimmune diseases in affected families. JAMA Intern Med. 2015;175(9):1518-1526.
Last reviewed March 2017 by EBSCO Medical Review Board Michael Woods, MD, FAAP
Last Updated: 11/9/2015

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