Scleroderma is a chronic autoimmune disease that was first described in the 18th century. The term scleroderma means "hard skin," which describes thickening of the skin from increased deposits of collagen.

There are two main types of scleroderma with a subset called CREST:
1. Localized scleroderma affects the skin in limited areas and the musculoskeletal system.
2. Systemic sclerosis causes more widespread skin changes and may be associated with internal organ damage in the lungs, heart and kidneys. It can cause arthritis, slow contractions in the gastrointestinal tract, muscle inflammation, dry eyes and dry mouth. Most people with scleroderma have cold-induced spasms of small blood vessels in their hands or feet, known as Raynaud’s phenomenon, which caused the fingers or toes to turn white or blue and may be painful.

Systemic sclerosis is associated with over-activation of the immune system, which normally functions to protect the body against cancers and invading infections. This causes damage to cells that line small blood vessels, which in turn leads to the over-production of scar tissue.

In most cases, the cause of scleroderma is unknown. However, in a small minority of cases, scleroderma or scleroderma-like illnesses are associated with exposure to certain toxins or as a complication of bone marrow transplants. Scleroderma is not contagious and is rarely inherited.

Scleroderma affects women more than men and adults more than children and there are 10-20 new cases are diagnosed per million people each year. The five-year survival rate is 80 to 85%. The lung, heart and kidney can become damaged, and are the most frequent causes of severe disability and death. Many people have decreased hand function because of joint disfigurement or finger ulcers.

Diagnosis of scleroderma is based on clinical history and physical findings. Diagnosis may be delayed in those without significant skin thickening. Laboratory, X-ray and pulmonary function tests determine the extent and severity of internal organ involvement.

CREST is a form of Systemic Sclerosis (scleroderma) which is characterized by:
Calcinosis (calcium deposits), usually in the fingers;
Raynaud's;
Loss of muscle control of the Esophagus, which can cause difficulty swallowing;
Sclerodactyly, a tapering deformity of the bones of the fingers; and
Telangiectasia, small red spots on the skin of the fingers, face, or inside of the mouth.

It takes only two of the five CREST symptoms for a diagnosis of CREST (either "pure" or "plus") to be made. For example, a patient with Calcinosis and Raynaud's would have CREST (which for precision may also be written as CRest, but it is CREST nonetheless.)

PureCREST is diagnosed when patients have two or more symptoms of CREST but they do not meet the criteria for either Limited or Diffuse Scleroderma. That is, they must not have tight skin above their wrists, and if there is tight skin on their fingers, they must not have either pitting digital ulcers or lung fibrosis.

Plus CREST is when CREST symptoms appear along with another form of Scleroderma. It is referred to as "Limited Scleroderma plus CREST" or "Diffuse Scleroderma plus CREST.", for example. A person may also have any other autoimmune disease "plus CREST."

Although some doctors still believe CREST is a useful subcategory, the existing research studies have been unable to predict consistently how (or whether) the disease will progress to Diffuse Systemic Scleroderma in any specific individual.

Conventional Treatment
There is no known cure for scleroderma. No treatment has been scientifically proven to alter the overall course of the disease, although d-penicillamine is commonly used for this purpose and may be of some value.

There are a number of effective organ-specific treatments for scleroderma. Raynaud’s phenomenon may be helped by calcium channel blockers. Declining renal function and hypertension are often treated with drugs. Esophageal damage from reflux of stomach contents can be treated with acid-reducing drugs. Antibiotics, special diets and medication can improve absorption of nutrients in people who have abnormalities of their intestines. Musculoskeletal pain may respond to nonsteroidal anti-inflammatory agents. Lung inflammation may be treated with cyclophosphamide.