The Different Types of Scleroderma (Systemic Sclerosis)

There are two main types or classes of scleroderma: localized scleroderma, which affects only certain parts of the body and includes linear scleroderma and morphea, and systemic sclerosis, which affects the entire body.

Scleroderma is a symptom of several rheumatic diseases (conditions characterized by inflammation and pain in the muscles, joints, or fibrous tissue) and connective tissue diseases. Many rheumatologists refer to the overall disease process as systemic sclerosis and the skin involvement as scleroderma. Scleroderma literally means "hard skin," derived from the Greek words sklerosis (which means hardness) and derma (which means skin).

An arm showing skin lesions caused by Scleroderma
AVM / Wikimedia Commons / CC BY 3.0

Some types of scleroderma have a limited process, which primarily makes the skin hard and tight. Other types are more complicated, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Localized Scleroderma

Localized types of scleroderma affect the skin and nearby tissues, and sometimes the muscle below. Internal organs are not affected, but the effects may be serious and disabling.

Localized scleroderma can never progress to the systemic type of the disease. These types can improve over time, but the skin changes that occur while the disease is active can be permanent.

There are two types of localized scleroderma:

  • Morphea: Reddish patches of skin that thicken into firm oval-shaped areas are distinctive features of the morphea type of localized scleroderma. The centers of the patches are ivory, with violet borders. The patches can occur on the chest, stomach, back, face, arms, and legs. The patches typically only minimally sweat and have little hair growth. Morphea can be localized (limited to one or several patches, ranging from half-inch to 12 inches in diameter) or generalized (skin patches are hard and dark and spread over larger areas of the body). Morphea generally fades out in three to five years, but dark skin patches can remain and, though rare, muscle weakness may remain as well.
  • Linear: A distinctive single line or band of thickened, abnormally colored skin typically characterizes the linear type of localized scleroderma. The line typically runs down an arm or leg, but can run down the forehead.

Systemic Sclerosis

Systemic sclerosis not only affects the skin but also involves blood vessels and major organs.

CREST Syndrome

Systemic sclerosis often involves a group of symptoms known as CREST syndrome.

Features of CREST syndrome:

  • Calcinosis: Formation of calcium deposits in the connective tissue
  • Raynaud's phenomenon: The blood vessels of the hands or feet contract in response to triggers, like cold or anxiety
  • Esophageal dysfunction: Impaired function of esophageal smooth muscles
  • Sclerodactyly: Thick and tight skin on your fingers due to deposits of excess collagen within skin layers
  • Telangiectasias: Small red spots on the hands and face caused by swelling of tiny blood vessels

A late complication of CREST can be elevated pulmonary artery pressure. Monitoring for this condition is recommended, typically by pulmonary function testing and/or echocardiogram.

Limited Scleroderma

Systemic sclerosis is divided into two categories, limited and diffuse. Limited scleroderma typically has a gradual onset and is restricted to certain areas of the skin, such as fingers, hands, face, lower arms, and legs.

You can experience Raynaud's phenomenon for years before skin thickening is evident. Sometimes skin problems affect much of the body, improving over time, and leaving only the face and hands with tight, thickened skin. Calcinosis and telangiectasias often follow.

Limited scleroderma is sometimes referred to as CREST syndrome because of the predominance of CREST symptoms in this condition.

Diffuse Scleroderma

Diffuse scleroderma typically has a sudden onset. Skin thickening develops quickly and covers much of the body, typically in a symmetric pattern. Major internal organs can be damaged. Symptoms common with diffuse scleroderma include:

  • Tiredness
  • Loss of appetite or loss of weight
  • Joint swelling
  • Joint pain

Skin can swell, appear shiny, and feel tight and itchy. The damage of diffuse scleroderma occurs over a period of a few years. After about three to five years, the condition stabilizes. During this phase, there is little apparent progression and symptoms subside—but gradually, skin changes begin again. A phase recognized as softening occurs, during which less collagen is made and the body rids itself of excess collagen.

The most recent areas of thickened skin are reversibly softened. Sometimes the skin returns to normal, and some people develop thin and fragile skin.

The most severe effects of this type of scleroderma are complications affecting the kidneys, lungs, heart, and digestive tract. Less than one-third of patients with the diffuse type of scleroderma develop severe complications of these organs.

Systemic Sclerosis Sine Scleroderma

Some recognize systemic sclerosis sine scleroderma as a third category of systemic sclerosis. This form affects the blood vessels and internal organs, but not the skin.

A Word From Verywell

It's important to understand the type of systemic sclerosis you're diagnosed with as you consider the effects of your treatment. Although there isn't a cure for systemic sclerosis or scleroderma, there are options available to help you manage your condition, reduce symptoms, and continue to live well.

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  2. Shah AA, Wigley FM. My approach to the treatment of scleroderma. Mayo Clin Proc. 2013;88(4):377-93. doi:10.1016/j.mayocp.2013.01.018

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