Other Names for this Disease
- Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
- Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
- CREST syndrome
- Limited cutaneous systemic scleroderma
What causes CREST syndrome?
How is CREST syndrome diagnosed?
How might CREST syndrome be treated?
In people with CREST syndrome, the immune system appears to stimulate cells called fibroblasts to produce excess amounts of collagen. Normally, fibroblasts synthesize collagen to help heal wounds, but in this case, the protein is produced even when it's not needed, forming thick bands of connective tissue around the cells of the skin, blood vessels and in some cases, the internal organs.
Although an abnormal immune system response and the resulting production of excess collagen appears to be the main cause of limited scleroderma, researchers suspect that other factors may play a role, including: genetic factors, pregnancy, hormones, and environmental factors.
A blood sample can be tested for antibodies that are frequently found in the blood of people with limited scleroderma. But this isn't a definitive test because not everyone with limited scleroderma has these antibodies.
Sometimes doctors take a small sample of skin that's then examined under a microscope in a laboratory. Biopsies can be helpful, but they can't definitively diagnose limited scleroderma either.
Along with a blood test and skin biopsy, additional tests to identify lung, heart or gastrointestinal complications may also be conducted.
For pain management, studies have shown that oxycodone is effective and safe for pain due to severe skin ulcers, while topical lidocaine helps reduce pain of digital ulcers in individuals with systemic scleroderma.
There are also some lifestyle changes and home remedies that may be helpful for some individuals with CREST syndrome. To reduce Raynaud's symptoms, individuals may consider wearing gloves or mittens outdoors when the weather is cool, and indoors when reaching into the freezer, for example. To maintain the body's core temperature, individuals may dress in layers and wear a hat or scarf, thermal socks, and well-fitting boots or shoes that don't cut off the circulation. Individuals who smoke should talk to their doctor about the best ways to quit. Nicotine constricts the blood vessels, making Raynaud's phenomenon worse. Individuals who have difficulty swallowing may consider choosing soft, moist foods and chewing food well. To minimize acid reflux individuals may eat small, frequent meals; avoid spicy or fatty foods, chocolate, caffeine, and alcohol; and avoid exercising immediately before or after eating. Sitting upright for a couple of hours after a meal may also help. To help keep skin soft, individuals may avoid harsh soaps and detergents, while choosing gentle skin cleansers and bath gels with added moisturizers. Individuals may also consider bathing less frequently and taking brief baths and showers, using warm rather than hot water. Moisture levels in the home may be improved by using a humidifier to ease skin and breathing symptoms.
For additional information about how CREST syndrome may be treated, the following article from eMedicine may be helpful: http://emedicine.medscape.com/article/1064663-treatment#showall
The information provided here is for general educational purposes only. Individuals interested in learning about specific treatment options for themselves or family members should speak with their healthcare provider.
- Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). 2010; http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp. Accessed 12/7/2011.
- Mayo Clinic Staff. Limited Scleroderma (CREST syndrome). MayoClinic.com. 2011; http://www.mayoclinic.com/health/crest-syndrome/DS00580. Accessed 12/7/2011.
- Makover ME. CREST syndrome. MedlinePlus. 2011; http://www.nlm.nih.gov/medlineplus/ency/imagepages/19507.htm. Accessed 12/7/2011.
- Jeanie C Yoon. CREST syndrome. eMedicine. September 22, 2009; http://emedicine.medscape.com/article/1064663-overview. Accessed 8/11/2011.
- Limited scleroderma (CREST syndrome). Mayo Clinic. June 2, 2011; http://www.mayoclinic.com/health/crest-syndrome/DS00580. Accessed 8/10/2011.
- Giuggioli D, Manfredi A, Colaci M, Ferri C. Oxycodone in the long-term treatment of chronic pain related to scleroderma skin ulcers. Pain Medicine. October 2010; 11(10):1500-1503. http://www.ncbi.nlm.nih.gov/pubmed/20456083. Accessed 8/11/2011.
- Ozgocmen S, Kaya A, Coskun BK. Topical lidocaine helps reduce pain of digital ulcers in systemic sclerosis (scleroderma). Clinical Rheumatology. May 2006; 25(3):378-379. http://www.ncbi.nlm.nih.gov/pubmed/16211339. Accessed 8/11/2011.