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Scleroderma

Definition

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

Alternative Names

Progressive systemic sclerosis; Systemic sclerosis; Limited scleroderma; CREST syndrome; Localized scleroderma; Morphea - linear; Raynaud's phenomenon - scleroderma

Causes

Scleroderma is a type of autoimmune disorder. In this condition, the immune system mistakenly attacks and damages 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. 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 undifferentiated connective tissue disease or overlap syndrome.

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 cold temperatures (Raynaud phenomenon)
  • Stiffness and tightness of skin of fingers, hands, forearm, and face
  • Hair loss
  • Skin that is darker or lighter than normal
  • 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:

Exams and Tests

The health care provider will do a complete physical exam. The exam may show:

  • Tight, thick skin on the fingers, the face or elsewhere.
  • The skin at the edge of the fingernails may be looked at with a lighted magnifying glass for abnormalities of the small blood vessels.
  • The lungs, heart and abdomen will be examined for abnormalities.

Your blood pressure will be checked. Scleroderma can cause small blood vessels in the kidneys to become narrowed. Problems with your kidneys can lead to high blood pressure and decrease function of the kidney.

Blood and urine tests may include:

Other tests may include:

Treatment

There is one no specific treatment for scleroderma. Your provider will assess the extent of disease in the lungs, kidneys, heart, and gastrointestinal tract.

You will be prescribed medicines and other treatments to control your symptoms and prevent complications.

Medicines used to treat scleroderma include:

  • Corticosteroids such as prednisone: Doses above 10 mg per day are not recommended.
  • Drugs that suppress the immune system such as methotrexate or mycophenolate.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) for short periods.
  • Hydroxychloroquine to treat arthritis.

Other treatments for specific symptoms may include:

  • Treatments to improve Raynaud phenomenon: These include medicines, gloves to keep the hands warm, and not smoking.
  • Medicines for heartburn or swallowing problems, such as omeprazole.
  • Blood pressure medicines, such as ACE inhibitors, for high blood pressure or kidney problems.
  • Light therapy to relieve skin thickening.
  • Medicines to improve lung function, such as bosentan and sildenafil.

Treatment often involves physical therapy as well.

Support Groups

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

Outlook (Prognosis)

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

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

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

When to Contact a Medical Professional

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

Prevention


Images

Raynaud's phenomenon
CREST syndrome
Sclerodactyly
Telangiectasia

References

Mendoza FA, Nagle SJ, Lee JB, Jimenez SA. A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol. 2012;39:1241. PMID: 22467932 www.ncbi.nlm.nih.gov/pubmed/22467932.

Walker KM, Pope J; participating members of the Scleroderma Clinical Trials Consortium (SCTC); Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails--a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012;42(1):42-55

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: Elsevier Saunders; 2012:chap 83.

Varga J. Systemic sclerosis (scleroderma). In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 267.

Review Date: 
1/16/2016
Reviewed By: 
Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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