Scleroderma

Overview

Definition

Scleroderma is a lifelong disease. It causes damage to the structures that support and protect the body. It can cause skin and other structures to thicken and stiffen. This can happen in the skin, joints, and internal organs.

There are 3 types:

  • Localized—Involves the skin and other parts. The areas are not near each other.
    • Morphea—Made up of local patches.
    • Linear—Single line or band of skin that's thickened or discolored
  • Systemic (sclerosis)—Over a wider area of skin and organs (mainly the lungs). This type is more serious and can lead to death. Raynaud phenomenon (RP) is related to this type.
    • Limited—Sometimes called CREST syndrome:
      • Skin thickening happens slowly. This happens mostly on the hands, forearms, feet, and lower legs.
      • May progress to the organs.
    • Diffuse—
      • Happens quicker. It causes the skin to thicken throughout the body.
      • May progress to the organs.
    • Sclerosis sine scleroderma—Involves the organs, not the skin. This type is very rare.
  • Overlap syndrome—May involve features of scleroderma and other connective tissue syndromes.
  • Undifferentiated—Involves RP and some features of the systemic type.

Causes

The body makes too much collagen and other proteins. This causes changes in the skin, tissues, and organs. It’s not clear why this happens. The immune system may be too active, leading to the overproduction.

Risk Factors

Scleroderma is more common in women.

The most common age groups are:

  • Morphea—20-40 years old
  • Linear—mainly children
  • Systemic—30-50 years old

Your chances are also higher if you:

  • Have others in your family with the same types of problems
  • Are exposed to chemicals at work

SymptomsandDiagnosis

Symptoms

Localized

Problems are contained to the skin. It doesn’t involve the organs. The skin problems may go away on their own. It may take a few months or years. For some, damage can be permanent. Skin problems may involve:

  • Hard patches on the skin, most often on the face or trunk—morphea
  • Lines of plaques or thickened skin
  • May spread to muscles or bones under the skin—linear
  • Muscle and joint aches, and fatigue—morphea

Systemic

Systemic disease may be limited or diffuse. The limited type can start slowly. Most often, it involves RP. Cold or emotional stress cause swelling, numbness, and color changes in the fingers and toes. Over time, the skin may thicken.

Raynaud Phenomenon Symptom
Low blood flwo to fingers, vasoconstriction
Copyright © Nucleus Medical Media, Inc.

The diffuse type comes on faster. It spreads to the skin and organs. Other problems of this type:

  • The skin thickens and gets harder
  • Joint and muscle pain—there may also be stiffness and swelling
  • Breathing problems
  • Swallowing problems
  • Blood vessels thicken and swell

The diffuse type can cause problems all over the body. This may involve:

  • Skin sores
  • Bleeding
  • Interstitial lung disease
  • Pulmonary hypertension
  • Heart rhythm problems
  • Heart failure
  • Kidney failure
  • High blood pressure

Diagnosis

The doctor will ask about your symptoms and health history. Your answers and a skin exam will point to scleroderma. You may also have:

  • A physical exam
  • Blood tests
  • Biopsies are taken of the skin and tissues
  • Imaging tests:
    • X-ray
    • MRI scan
    • CT scan
    • Echocardiogram

Treatments

Treatment

There is no cure for scleroderma. Care focuses on easing symptoms.

Your doctor may advise these or other medicines to treat:

  • Pain and swelling
  • The immune system
  • Gastroesophageal reflux disease—GERD
  • High blood pressure
  • Narrowed blood vessels

Other care may involve:

  • Physical therapy and exercise to help with circulation, flexibility, and strength
  • Phototherapy—to thin out the skin
  • Changes to control GERD such as eating smaller meals or raising the head of your bed
  • Quitting smoking to improve blood flow

Prevention

There is no way to prevent scleroderma because the exact cause is unknown.

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

Edits to original content made by Denver Health.

a (Systemic Sclerosis)

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

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;54(3):440-447.

Localized scleroderma. EBSCO DynaMed Plus website. Available at:  http://www.dynamed.com/topics/dmp~AN~T114314/Localized-scleroderma . Updated June 7, 2017. Accessed June 20, 2018.

Mathai SC, Girgis RE, Fisher MR, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007;29(3):469-475.

Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity. 2005;38(3):219-223.

Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: https://www.niams.nih.gov/health-topics/scleroderma. Accessed June 20, 2018.

Systemic sclerosis. EBSCO DynaMed Plus website. Available at:  http://www.dynamed.com/topics/dmp~AN~T116347/Systemic-sclerosis . Updated March 22, 2018. Accessed June 20, 2018.

What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageNavigator/patients%5Fwhatis.html#.Wy58BVVKhxA. Accessed June 20, 2018.

11/9/2015 DynaMed Plus Systematic Literature Surveillance  http://www.dynamed.com/topics/dmp~AN~T116347/Systemic-sclerosis : Kuo CF, Grainge MJ, Valdes AM, et al. Familial aggregation of systemic lupus erythematosus and coaggregation of autoimmune diseases in affected families. JAMA Intern Med. 2015;175(9):1518-1526.