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Scleroderma
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Scleroderma

(Systemic Sclerosis)

Pronounced: Sclare-oh-DER-mah

En Español (Spanish Version)More InDepth Information on This Condition

Definition

Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:

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—patients may have features of scleroderma as well as features of other autoimmune syndromes

Causes

Overproduction of collagen and other connective tissue proteins is the main feature of scleroderma, but it is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production. There is also evidence that scleroderma results from diffuse vascular abnormalities that may be related to this immune dysfunction.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Your risk of scleroderma increases if:

  • You are female
  • You are 30 to 50 years old
  • You have certain occupational exposures (such as polyvinyl chloride or silica dust)
  • You have a genetic predisposition

Symptoms

Localized scleroderma—Symptoms are usually restricted to the skin and 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 (swelling, tingling, numbness, blue and white color, and pain of fingers and toes brought on by cold or emotional distress) and progress over the years to thickened skin.

Raynaud's Phenomenon Symptom

Low blood flwo to fingers, vasoconstriction

© 2008 Nucleus Medical Art, Inc.

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. Prominent complications include:

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam. Diagnosis is based on changes in the skin. Other tests may include:

  • Blood tests
  • Esophagus motility study
  • Imaging tests—plain x-rays , CT scan , or MRI scan to look at internal organs
  • Biopsies of skin and other tissues—removing a sample of tissue for testing

Treatment

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

Joints and Muscles

Esophagus and Digestive Tract

  • Medications 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

Raynaud's Phenomenon

  • Calcium channel blockers, like Nifedipine–to dilate blood vessels in the extremities
  • Proper shelter and clothing to avoid cold
  • Not smoking

Restricted Mobility

  • Physical therapy and exercise to maintain circulation, joint flexibility, and muscle strength

Prevention

There are no guidelines for preventing scleroderma because the cause is unknown.

RESOURCES:

Scleroderma Foundation
http://www.scleroderma.org

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

CANADIAN RESOURCES:

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

The Arthritis Society
www.arthritis.ca

References:

Arthritis Foundation website. Available at: http://www.arthritis.org .

Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. 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 Mar;54(3):440-7.

Mathai et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J . 2006 Nov 1.

Mayo Foundation for Medical Education and Research website. Available at: http://www.mayo.edu/ .

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 May;38(3):219-23.



Last reviewed January 2008 by Ross Zeltser, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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