Limited scleroderma (CREST syndrome)

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Complications

By Mayo Clinic staff

The visible signs of limited scleroderma — tight, thick skin on your fingers, hands and face — can affect the way you feel about your appearance; make everyday tasks, such as opening a jar or shaving, more difficult; and even affect your speech. But the most serious complications tend to occur beneath your skin.

Gastrointestinal problems
Changes in the functioning of esophageal muscles can cause difficulty swallowing and chronic heartburn. When limited scleroderma affects your intestine, you may experience:

  • Constipation or diarrhea
  • Bloating after meals
  • Weight loss
  • Malnutrition

Ulcers on fingers and toes
Severe Raynaud's phenomenon can obstruct blood flow to your extremities and may cause ulcers of the fingers and toes. These ulcers can be difficult to heal. Additionally, abnormal or narrowed blood vessels combined with severe Raynaud's phenomenon can lead to gangrene of fingers or toes, which may require amputation.

Lung damage
Limited scleroderma can cause a variety of problems with your lungs, including:

  • Interstitial lung disease. This disorder occurs when excess collagen collects in the tissue between the lungs' air sacs, making the lung tissue stiffer and less able to work properly.
  • Pulmonary hypertension. Increased pressure in the arteries between your heart and lungs makes the heart work harder and eventually weakens it.

Heart problems
Scarring of heart tissue can lead to abnormal heart rhythms (arrhythmias) and, in rare cases, to an inflamed heart muscle (myocarditis).

Dental problems
Severe tightening of facial skin can cause your mouth to become smaller and narrower, putting you at risk of serious dental problems. A measurably smaller mouth makes it hard to brush your teeth or have them professionally cleaned, increasing your risk of tooth decay. And because people with limited scleroderma often don't produce normal amounts of saliva, the risk of decay increases even more. In addition, acid reflux can destroy tooth enamel, and changes in gum tissue may cause your teeth to become loose or even fall out.

Dry eyes and mouth
Many people with limited scleroderma experience very dry eyes and mouth.

References
  1. Habif TP. Scleroderma. In: Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th ed. Edinburgh, U.K.; New York, N.Y.: Mosby Elsevier; 2010. http://www.mdconsult.com/books/page.do?sid=1149581384&eid=4-u1.0-B978-0-7234-3541-9..00026-2--s0540&isbn=978-0-7234-3541-9&type=bookPage§ionEid=4-u1.0-B978-0-7234-3541-9..00026-2--s0605&uniqId=239740754-5. Accessed April 23, 2011.
  2. Ferri FF. Scleroderma (progressive systemic sclerosis). In: Ferri FF. Ferri's Clinical Advisor 2011: Instant Diagnosis and Treatment. Philadelphia, Pa.: Mosby Elsevier; 2011. http://www.mdconsult.com/books/page.do?eid=4-u1.0-B978-0-323-05610-6..00028-7--sc29005&isbn=978-0-323-05610-6&sid=1149581384&type=bookPage§ionEid=4-u1.0-B978-0-323-05610-6..00028-7--s29225&uniqId=239740754-5#4-u1.0-B978-0-323-05610-6..00028-7--s29225. Accessed April 23, 2011.
  3. Systemic sclerosis. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merckmanuals.com/professional/sec04/ch032/ch032h.html. Accessed April 23, 2011.
  4. Steen VD. The many faces of scleroderma. Rheumatic Disease Clinics of North America. 2008;34:1.
  5. Autoimmune connective tissue disease. In: Bope ET, et al. Conn's Current Therapy. Philadelphia, Pa.: Saunders Elsevier; 2011. http://www.mdconsult.com/books/page.do?eid=4-u1.0-B978-1-4377-0986-5..00007-7--sc0040&isbn=978-1-4377-0986-5&sid=1149586698&type=bookPage§ionEid=4-u1.0-B978-1-4377-0986-5..00007-7--s0715&uniqId=239740754-7#4-u1.0-B978-1-4377-0986-5..00007-7--s0715. Accessed April 23, 2011.
  6. Handout on health: Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases. http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp. Accessed April 23, 2011.
  7. Agarwal SK, et al. Genetics and genomic studies in scleroderma (systemic sclerosis). Rheumatic Disease Clinics of North America. 2008;34:17.
DS00580 June 2, 2011

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