Munson Health
 
Scleroderma

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by Alan R

(Systemic Sclerosis)

 

Symptoms

Localized Scleroderma

Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:
  • 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)

Systemic Scleroderma

This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with Raynaud's phenomenon . This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.
Raynaud's Phenomenon Symptom
Low blood flwo to fingers, vasoconstriction
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Diffuse scleroderma comes on more suddenly and can progress to involve not only the skin but the internal organs. Other symptoms of diffuse scleroderma may include:
  • 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
Complications of diffuse scleroderma can affect virtually every system of the body. Common complications include:
 

Treatment

There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms.

Joints and Muscles

  • Aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Corticosteroids

Skin and Internal Organs

Kidney Problems and/or High Blood Pressure

  • ACE inhibitors, or other antihypertensive drugs
  • Antineoplastic agents
  • Vasodilators

Raynaud's Phenomenon

  • Calcium channel blockers
  • Proper shelter and clothing to avoid cold
  • Not smoking

Restricted Mobility

Physical therapy and exercise are important to maintain circulation, joint flexibility, and muscle strength .
 

RESOURCES

Scleroderma Foundation
http://www.scleroderma.org

Scleroderma Research Foundation
http://www.srfcure.org

 

CANADIAN RESOURCES

Arthritis Society
http://www.arthritis.ca

Scleroderma Society of Canada
http://www.scleroderma.ca

 

References


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.


Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity. 2005;38:219-223.


Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov/Health%5FInfo/Scleroderma/default.asp . Updated August 2012. Accessed August 21, 2013.


Systemic sclerosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed.ebscohost.com/about/about-us . Updated August 5, 2013. Accessed August 21, 2013.


What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageNavigator/patients%5Fwhatis.html . Accessed August 21, 2013.

 

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