by EBSCO Medical Review Board
(Systemic Sclerosis)

Definition

Scleroderma is a disease that affects the whole body. It can cause the skin, joints, and internal organs to thicken and stiffen.

There are 2 main types:

  • Localized scleroderma affects the skin (most common)
  • Systemic scleroderma can affect many body parts or systems

Causes

The exact cause is not known. It may be due to genetics, the environment, or a problem with how the immune system works.

Risk Factors

This health problem is more common in women. It often starts between 20 and 50 years of age.

The risk of this problem is raised in people who have other family members with lupus.

Symptoms

Symptoms differ from person to person and by type of scleroderma.

A person with the localized type may have:

  • Patches of thick, hard skin that are oval in shape
  • Lines of thickened skin on the arms, legs, or face

A person with the systemic type may have:

  • Numbness, discomfort, or a change in skin color in the fingers and toes
  • Tight, thickened, or shiny skin
  • Muscle pain
  • Diarrhea or problems passing stool
Raynaud Phenomenon Symptom
Low blood flwo to fingers, vasoconstriction
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Diagnosis

The doctor will ask about your symptoms and health history. A physical exam will be done.

These tests may also be done:

  • Blood tests to look for antibodies linked to scleroderma
  • A skin biopsy

Images may be taken of structures in your body. This can be done with:

Treatment

There is no cure. The goal of treatment is to manage the symptoms a person is having. Choices are:

  • Medicines to:
    • Ease pain and swelling
    • Treat skin changes
    • Widen blood vessels
    • Suppress the immune system
    • Reduce stomach acid
  • Physical therapy to help with strength and flexibility
  • Occupational therapy to learn how to do day-to-day activities
  • Phototherapy to thin out the skin

Prevention

There are no known guidelines to prevent this health problem.

RESOURCES

Scleroderma Foundation  http://www.scleroderma.org 

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

CANADIAN RESOURCES

Arthritis Society  http://www.arthritis.ca 

Scleroderma Canada  http://www.scleroderma.ca 

References

Denton CP. Advances in pathogenesis and treatment of systemic sclerosis. Clin Med (Lond). 2015 Dec;15 Suppl 6:s58-63.

Localized scleroderma. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/localized-scleroderma. Accessed December 1, 2020.

Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: https://www.niams.nih.gov/health-topics/scleroderma. Accessed December 1, 2020.

Systemic sclerosis. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/systemic-sclerosis. Accessed December 1, 2020.

What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageNavigator/patients%5Fwhatis.html#.Wy58BVVKhxA. Accessed December 1, 2020.

Revision Information

  • Reviewer: EBSCO Medical Review Board Marcin Chwistek, MD
  • Review Date: 09/2020
  • Update Date: 04/23/2021