- Localized scleroderma (also known as morphea)—usually affects only the skin in isolated parts of the body. This form is less serious.
- Systemic scleroderma—affects widespread areas of skin and/or internal organs, most often the lungs (Certain categories of this form of scleroderma are more serious and can be fatal.)
- Overlap syndrome—may involve features of scleroderma and features of other autoimmune syndromes
- Sex: Female
- Age: 30-50 years
- Occupational exposures (such as, polyvinyl chloride or silica dust)
- Genetic predisposition
- Hard patches on the skin, most often on the face or trunk (morphea)
- Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)
|Raynaud's Phenomenon Symptom|
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- Diffuse thickening and hardening of the skin
- Joint and muscular pain, stiffness, and swelling
- Problems with breathing, swallowing, and digesting food due to thickening and hardening of lung, esophagus, bowel tissues
- Inflammation and thickening of large and small blood vessels
- Blood tests
- Esophagus motility study
- X-ray—a test that uses radiation to take a picture of structures inside the body, especially bones
- MRI scan—a test that uses magnetic waves to make pictures of structures inside the body
- CT scan—a type of x-ray that uses a computer to make pictures of structures inside the body
- Biopsies of skin and other tissues—removal of a sample of tissue to test
Joints and Muscles
- Nonsteroidal anti-inflammatory drugs
- Corticosteroids, or prednisone (Deltasone, Orasone, Prednicen-M)
Esophagus and Digestive Tract
- Medicines to limit acid production in the stomach
- Small, frequent meals
- Sleeping with your head elevated to avoid acid reflux
Skin and Internal Organs
Kidney Problems and/or High Blood Pressure
- Angiotensin-converting enzyme (ACE) inhibitors and other antihypertensive drugs
- Gleevec (imatinib mesylate)—already approved for the treatment of certain leukemias; used in several trials and may inhibit the production of fibrosis in scleroderma
- Pulmonary hypertension treatment (such as, bosentan, sildenafil)
- Calcium channel blockers, like nifedipine (Adalat, Procardia, Afeditab, Nifediac)—to dilate blood vessels in the extremities
- Proper shelter and clothing to avoid cold
- Not smoking
Scleroderma Foundation http://www.scleroderma.org
Scleroderma Research Foundation http://www.srfcure.org
Arthritis Society http://www.arthritis.ca
Scleroderma Society of Canada http://www.scleroderma.ca
Arthritis Foundation website. Available at: http://www.arthritis.org. Accessed July 9, 2009.
Firestein ED et al. Kelley’s Textbook of Rheumatology. 8th ed. Philadelphia: Saunders, 2008.
Habif TP. Clinical Dermatology. 4th ed. St. Louis: Mosby, 2004.
Kreuter A, Hyun J, et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440-447.
Mathai SC, Girgis RE, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007;29:469-475.
Mayo Foundation for Medical Education and Research website. Available at: http://www.mayo.edu. Accessed July 9, 2009.
The Merck Manual of Medical Information. 17th ed. Simon and Schuster, Inc; 2000.
Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity. 2005;38:219-223.
- Reviewer: Rosalyn Carson-DeWitt, MD
- Review Date: 09/2011 -
- Update Date: 09/01/2011 -