Types of Scleroderma (Systemic Sclerosis)

Scleroderma Is Not a Single Disease

Picture of a female patient's left arm, showing skin lesions caused by Scleroderma.
AVM/Wikimedia Commons/CC BY 3.0

Scleroderma literally means "hard skin", derived from the Greek words sklerosis (which means hardness) and derma (which means skin). Often thought of as a single disease, scleroderma is actually a symptom of a group of diseases that are complicated by an abnormal growth of connective tissue (tissue which supports the skin and internal organs). In fact, many rheumatologists refer to the disease as systemic sclerosis and the skin involvement as scleroderma.

Some types of scleroderma have a limited abnormal 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.

Scleroderma is considered both a rheumatic disease (conditions characterized by inflammation and pain in the muscles, joints, or fibrous tissue) and a connective tissue disease.

There are two main classes of scleroderma:

  • Localized Scleroderma (affects only certain parts of the body)
  • Systemic Sclerosis (affects the whole body)

Localized Scleroderma:

Localized types of scleroderma affect the skin and related tissues, and sometimes the muscle below. Internal organs are not affected. Localized scleroderma can never progress to the systemic type of the disease. Localized types can improve over time, but the skin changes that occur when the disease is active can be permanent. It may be serious and disabling.

There are two types of Localized Scleroderma: Morphea and Linear.


Reddish patches of skin that thicken into firm oval-shaped areas are distinctive of the Morphea type of Localized Scleroderma. The center of the patches are ivory, with violet borders. The patches can occur on the:

  • chest
  • stomach
  • back
  • face
  • arms
  • legs

The patches typically sweat little 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 3 to 5 years but people can have dark skin patches and, though rare, muscle weakness that remains.


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 skin but also involves blood vessels and major organs.

CREST Syndrome

People with Systemic Sclerosis often have all of the following symptoms, known as CREST syndrome, an acronym for:

  • Calcinosis (formation of calcium deposits in the connective tissue)
  • Raynaud's phenomenon (small blood vessels of hands or feet contract in response to cold or anxiety)
  • Esophageal dysfunction (impaired function of esophagus occurs when smooth muscles in the esophagus lose normal movement)
  • Sclerodactyly (thick and tight skin on fingers result from deposits of excess collagen within skin layers)
  • Telangiectasias (small red spots on the hands and face are caused by swelling of tiny blood vessels)

Systemic Sclerosis is divided into two categories, Diffuse and Limited.

Limited Scleroderma

Limited Scleroderma typically has gradual onset and is restricted to certain areas of the skin, such as:

  • fingers
  • hands
  • face
  • lower arms
  • legs

Raynaud's phenomenon is often experienced for years before skin thickening is evident. Others experience skin problems over much of the body that shows improvement over time, leaving only the face and hands with tight, thickened skin. Calcinosis and telangiectiasias often follow.

Limited Scleroderma is sometimes referred to as CREST syndrome because of the predominance of CREST symptoms in these patients.

Diffuse Scleroderma

Diffuse Scleroderma typically has 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 3 to 5 years, patients tend to stabilize, a phase when progression seems little 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 last areas thickened are reversibly softened. Some patient's skin returns to what seems like normal while the skin of others becomes thin and fragile. Patients with Diffuse Scleroderma face the most critical prognosis, if they develop severe complications in the:

  • kidneys
  • lungs
  • heart
  • digestive tract

Less than one-third of patients with the Diffuse type of Scleroderma develop severe complications in the aforementioned 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.


Scleroderma Handout On Health. NIAMS. February 2015.

Kelley's Textbook of Rheumatology. Ninth edition. Elsevier. Chapter 84 - Clinical Features and Treatment of Scleroderma.

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