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Scleroderma

Definition

Scleroderma is a disease that involves the buildup of scar-like tissue in the skin. It also damages the cells the line the walls of the small arteries.

Alternative Names

CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Mixed connective disease; Morphea - linear

Causes, incidence, and risk factors

 Scleroderma is a type of autoimmune disorder, in which the immune system mistakenly attacks and destroys healthy body tissue.

The cause of scleroderma is unknown. A buildup of a substance called collagen in the skin and other organs leads to the symptoms of the disease.

The disease most often affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.

Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis.These cases are referred to as mixed connective disease.

Symptoms

Some types of scleroderma affect only the skin, while others affect the whole body.

  • Localized scleroderma. Often affects only the skin on the hands and face. It develops slowly, and rarely spreads in the body or causes serious problems.
  • Systemic scleroderma, or sclerosis. May affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types limited disease (CREST syndrome) and diffuse disease.

Skin symptoms of scleroderma may include:

  • Fingers or toes that turn blue or white in response to hot and cold temperatures (Raynaud's phenomenon)
  • Hair loss
  • Skin hardness and thickening
  • Skin that is darker or lighter than normal
  • Stiffness, and tightness skin of fingers, hands, and forearm
  • Small white lumps beneath the skin that sometimes ooze a white substance that looks like toothpaste
  • Sores (ulcers) on the fingertips or toes
  • Tight and mask-like skin on the face

Bone and muscle symptoms may include:

  • Joint pain
  • Numbness and pain in the feet
  • Pain, stiffness, and swelling of fingers and joints
  • Wrist pain

Breathing problems may result from scarring in the lungs and can include:

Digestive tract problems may include:

Signs and tests

The health care provider will do a physical exam. The exam may show hard, tight, thick skin.

Your blood pressure will be checked. Scleroderma can cause small blood vessels in the kidneys to become inflamed. Problems with your kidneys can lead to high blood pressure.

Blood tests may include:

Other tests may include:

Treatment

There is no specific treatment for scleroderma.

Your doctor will prescribe medicines and other treatments to control your symptoms and prevent complications.

Medicines used to treat scleroderma include:

  • Corticosteroids
  • Drugs that suppress the immune system such as methotrexate and Cytoxan
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)

Other treatments for specific symptoms may include:

  • Medicines for heartburn or swallowing problems
  • Blood pressure medications (such as ACE inhibitors) for high blood pressure or kidney problems
  • Light therapy to relieve skin thickening
  • Medicines to improve breathing (bosentan)
  • Medications to treat Raynaud's phenomenon

Treatment usually also involves physical therapy.

Support Groups

Some people can benefit from attending a support group for people with scleroderma.

Expectations (prognosis)

In some people, symptoms develop quickly for the first few years and continue to get worse. However, in most people, the disease slowly gets worse.

People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to.

  • Cancer
  • Heart failure
  • Scarring of the lungs called pulmonary fibrosis
  •  High blood pressure in the lungs (pulmonary hypertension)
  • Kidney failure
  • Problems absorbing nutrients from food

Calling your health care provider

Call your health care provider if your symptoms become worse or new symptoms develop.

References

Varga J. Systemic Sclerosis (Scleroderma). In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap XX.

Varga J. Etiology and Pathogenesis of Scleroderma.. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, Pa: Saunders Elsevier;2012:chap 83.


Review Date: 2/21/2013
Reviewed By: Ariel D. Teitel, MD, MBA, Clinical Associate Professor of Medicine, NYU Langone Medical Center. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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