What Is Scleroderma? (also known as Systemic Sclerosis)
Scleroderma is a term that comes from the Greek for "hard skin." It's classified as both a connective tissue disorder and a rheumatic condition. In scleroderma, the skin gradually tightens and thickens or hardens. It loses its ability to stretch.

The disease usually affects the hands, face and feet.  However, tiny blood vessels throughout the body also may be affected.  This causes widespread damage to internal organs in the digestive, respiratory and circulatory systems.

Scleroderma can look very different in different people. There are many types of this condition.  Doctors generally classify scleroderma as either localized or systemic.  The classification depends on how much of the skin is affected.  The widespread form of the disease is often called systemic sclerosis and can be life-threatening.

An estimated 300,000 Americans have scleroderma. People in the United States are more likely to get the disease than people in Europe or Japan .  It varies across geographic regions.  No one race or ethnic group is affected more than any other. 

Women develop localized forms of the disease three times more than men.  More than 80% of people with systemic scleroderma are women aged 30 to 50.  Rarely, children can develop scleroderma.  Better treatments developed in the past two decades have led to improved longevity for people with systemic sclerosis.
Symptoms include:

  • Raynaud's phenomenon. This term refers to color changes (blue, white and red) that occur in fingers (and sometimes toes), often after exposure to cold temperatures. It occurs when blood flow to the hands and fingers is temporarily reduced.
  • Skin thickening, swelling and tightening. This is the problem that leads to the name "scleroderma". The skin may also become glossy or unusually dark or light in places. The disease can sometimes result in changes is personal appearance, especially in the face. When the skin becomes extremely tight, the function of the area affected can be reduced.
  • Enlarged red blood vessels on the hands, face and around nail beds (called "telangiectasias").
  • Calcium deposits in the skin or other areas.
  • High blood pressure from kidney problems.
  • Heartburn; this is an extremely common problem in scleroderma.
  • Other problems of the digestive tract such as difficulty swallowing food, bloating and constipation, or problems absorbing food leading to weight loss.
  • Shortness of breath.
  • Joint pain

 



What Causes Scleroderma?

The cause of scleroderma is not known. Genetic factors (different genes) appear be important in the disease. Although exposure to certain chemicals may play a role in some people having scleroderma, the vast majority of patients with scleroderma do not have a history of exposure to any suspicious toxins. The cause of scleroderma is likely quite complicated.

Who Gets Scleroderma?

Scleroderma is relatively rare. About 75,000 to 100,000 people in the U.S. have this disease; most are women between the ages of 30 and 50. Twins and family members of those with scleroderma or other autoimmune connective tissue diseases, such as lupus, may have a slightly higher risk of getting scleroderma. Children can also develop scleroderma, but the disease is different in children than in adults.

How is Scleroderma Diagnosed?

Diagnosis can be tricky because symptoms may be similar to those of other diseases. There is no one blood test or X-ray that can say for sure that you have scleroderma.To make a diagnosis, a doctor will ask about the patient's medical history, do a physical exam and possibly order lab tests and X-rays and look for the above symptoms.

How is Scleroderma Treated?

While some treatments are effective in treating some aspects of this disease, there is no drug that has been clearly proven to stop, or reverse, the key symptom of skin thickening and hardening. Doctors aim to curb individual symptoms and prevent further complications with a combination of drugs and self-care.

Note: Please see your physician/rheumatologist if you have any of the above symptoms.

Source: http://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Scleroderma_(also_known_as_systemic_sclerosis)/