Limited scleroderma (CREST syndrome)

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Symptoms

By Mayo Clinic staff

While some varieties of scleroderma occur rapidly, signs and symptoms of limited scleroderma usually develop gradually. They include:

  • Tight, hardened skin. In limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and throat. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.
  • Raynaud's phenomenon. This condition occurs when small blood vessels in your fingers and toes spasm in response to cold or emotional stress, blocking the flow of blood. In most people, the skin turns white before becoming blue, cold and numb. When circulation improves, the skin usually reddens and may throb or tingle. Raynaud's phenomenon is often one of the earliest signs of limited scleroderma, but many people have Raynaud's only and never develop scleroderma.
  • Red spots or lines on skin. These small red spots or lines are caused by the swelling of tiny blood vessels (telangiectasias) near the skin's surface. They are not painful and occur primarily on the hands and face.
  • Bumps under the skin. Limited scleroderma may cause tiny calcium deposits (calcinosis) to develop under your skin, mainly on your elbows, knees and fingers. You can see and feel these deposits, which sometimes may be tender or become infected.
  • Swallowing difficulties. People with limited scleroderma commonly experience problems with their esophagus — the tube that connects the mouth and stomach. Poor functioning of the muscles in the upper and lower esophagus can make swallowing difficult and allow stomach acids to back up into the esophagus, leading to heartburn, inflammation and scarring of esophageal tissues.
References
  1. Handout on health: Scleroderma. National Institute of Arthritis and Musculoskeletal Diseases. http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp. Accessed March 18, 2009.
  2. Steen VD. The many faces of scleroderma. Rheumatic Disease Clinics of North America. 2008;34:1.
  3. Wigley FM. Scleroderma. In: Goldman L, et al. Cecil Medicine. 23rd ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/126871837-5/818551240/1492/1032.html#4-u1.0-B978-1-4160-2805-5..50293-7--cesec9_13002. Accessed March 19, 2009.
  4. Raynaud's phenomenon. National Institute of Arthritis and Musculoskeletal and Skin Diseases. http://www.niams.nih.gov/Health_Info/Raynauds_Phenomenon/default.asp. Accessed March 18, 2009.
  5. Interstitial lung disease and pulmonary fibrosis. American Lung Association. http://www.lungusa.org/site/apps/nlnet/content3.aspx?c=dvLUK9O0E&b=2060161&content_id={4350F20F-98E4-403B-A33B-68B20A3C2FBA}&notoc=1. Accessed March 19, 2009.
  6. Pulmonary hypertension. National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_what.html. Accessed March 19, 2009.
  7. Denton CP. Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults. http://www.uptodate.com/home/index.html. Accessed March 20, 2009.

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June 2, 2009

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