What Are the Different Types of Scleroderma?

Scleroderma Is an Autoimmune Connective Tissue Disorder

Arthritic hands
Jodi Jacobson/E+/Getty Images

Scleroderma refers to a set of disorders with similar symptoms. These disorders are rheumatic diseases of the connective tissue. Scleroderma is thought to be an autoimmune disorder, meaning that the body attacks its own cells and damages them. What sets the body against itself isn't yet known.

Although scleroderma is not inherited, there may be a tendency to develop it in families that have a history of it or other rheumatic diseases.

Some cases of scleroderma have been linked to environmental factors such as exposure to certain chemicals or pollutants.

Scleroderma disorders affect around 300,000 people in the United States. About one-third of these individuals have scleroderma occurring throughout the body (called "systemic"), which is the most serious kind. Scleroderma can develop in every age group from infants to elderly, but most frequently begins between the ages of 25 to 55. Eighty percent of people affected by scleroderma are women.

Types of Scleroderma

There are two major types of scleroderma:

  • Localized scleroderma affects only the skin – includes localized and generalized morphea and linear scleroderma
  • Systemic scleroderma (systemic sclerosis) affects many parts of the body, including the skin, blood vessels, heart, lungs, kidneys, and other organs. Includes systemic limited scleroderma (CREST syndrome) and diffuse scleroderma

    Localized Scleroderma

    Usually only the skin is affected in localized scleroderma. There may be reddish patches of inflamed and discolored skin, as in morphea, or bands of thick, tight skin, as in linear scleroderma. In many people, the symptoms of localized scleroderma will improve or go away on their own over time.

    However, in some people scar tissue is left behind that may be disfiguring or painful.

    Systemic Scleroderma

    Systemic scleroderma is the most serious kind of scleroderma because it affects many parts of the body. It causes:

    • loss of circulation that can create painful skin ulcers on the fingers and toes
    • inflammation of the joints, causing pain
    • skin tightening on the fingers and toes reduces flexibility
    • skin tightening around the mouth can make eating and drinking difficult

    About 90% of people with systemic scleroderma experience Raynaud's phenomenon, in which the fingers and/or toes turn white and cold.

    Systemic scleroderma without skin symptoms is called sine sclerosis.

    Systemic Limited Scleroderma

    About two-thirds of the people with systemic scleroderma have systemic limited scleroderma, often called CREST syndrome:

    • Calcinosis - painful calcium deposits in the skin
    • Raynaud's phenomenon - fingers and/or toes turn white and cold
    • Esophageal dysfunction - problems with swallowing caused by internal scarring
    • Clerodactyly - skin tightening on the fingers and toes progressing to the hands and face, and sometimes the feet and neck
    • Elangiectasia - red spots on the hands, palms, forearms, face, and lips

    Diffuse Scleroderma

    Most people with diffuse scleroderma have extensive skin scarring (fibrosis) over large parts of the body, arms, and legs. Scarring in the digestive system can make eating and elimination difficult. Diffuse scleroderma may affect the kidneys, causing them to stop functioning (kidney failure). It may affect the lungs, causing high blood pressure in the lungs (pulmonary hypertension), and it may affect the heart, interfering with its function. People with diffuse scleroderma often feel chronically tired and have joint swelling and pain.

    Diagnosis of Scleroderma

    Correctly diagnosing scleroderma may take time because some doctors are not familiar with it, and they may not recognize it. Rheumatologists (arthritis specialists) and dermatologists (skin specialists) are the specialists most familiar with scleroderma and its symptoms. Blood tests and other special tests may be needed to confirm the diagnosis, see which organs in the body are affected, and determine which type of scleroderma is present.

    Treatment of Scleroderma

    Although there is no cure for scleroderma, many treatments are available to manage symptoms such as Raynaud's phenomenon, joint pain and stiffness, digestive problems, and lung and kidney problems. Some people with mild symptoms may not need medication. Some people may only take medication when their symptoms flare up. A scleroderma specialist will develop a treatment plan specific for each individual based on his symptoms.

    The Scleroderma Foundation's Medical Advisory Board has reviewed 19 scleroderma centers of excellence in the United States. Some individuals with scleroderma travel to these centers to be evaluated by specialists.


    "What Is Scleroderma?" Education & Resources. May 2008. Scleroderma Foundation. 16 May 2008.

    "What is Scleroderma?" Patient Information. Scleroderma Research Foundation. 16 May 2008.


    Continue Reading