Symptoms and Signs of Systemic Scleroderma

Medical Author:
Medically Reviewed on 9/14/2022

Doctor's Notes on Systemic Scleroderma

Systemic scleroderma (also called systemic sclerosis) is a chronic autoimmune disorder that affects the connective tissues of the skin and internal organs. Systemic scleroderma is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The scarring is due to the excess production of the protein collagen, which normally strengthens and supports connective tissues throughout the body.
Early symptoms of systemic scleroderma include

  • episodes of Raynaud phenomenon, in which the fingers and toes turn white or blue in response to cold temperature or other stresses; and
  • puffy or swollen hands before the skin thickens and hardens due to fibrosis.
  • Skin thickening usually occurs first in the fingers and may also involve the hands and face.

Other symptoms of systemic scleroderma include

  • open sores on the fingers,
  • painful bumps under the skin, or
  • small clusters of enlarged blood vessels just under the skin.

Fibrosis can also affect internal organs and can lead to damage or failure of the affected organs. The most commonly affected organs are the esophagus, heart, lungs, and kidneys. Symptoms of internal organ fibrosis due to systemic scleroderma may include

What is the treatment for systemic scleroderma?

Unfortunately, no treatments are available to cure or stop collagen overproduction. However, treatments are available to reduce symptoms; symptoms are what help determine treatment protocols. Protocols vary:

  • blood pressure medications to dilate blood vessels
  • Suppress immune system to reduce skin thickening
  • Antacids, antibiotics and fiber to reduce acid, diarrhea and constipation
  • Infection prevention with vaccines
  • Keep extremities protected from cold temperatures
  • Pain management – prescribed medications
  • Therapy to improve functions of hands, improve strength and mobility
  • Surgical treatments – stem cell transplants, organ transplants

Again, your symptoms can help decide which protocols your doctors may suggest.

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REFERENCE:

Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.