Dr. Abhrajit Ray

MD (CAL). MRCP (UK), MRCP (LONDON)
Radiant Medical Center, Kolkata
Visiting Consultant Physician & Rheumatologist
Fortis, Bellevue & Woodlands Hospitals

Scleroderma

Scleroderma (also known as systemic sclerosis) is a chronic disease that causes the skin to become thick and hard, a buildup of scar tissue, and damage to internal organs such as the heart and blood vessels, lungs, stomach and kidneys. The effects of scleroderma vary widely and range from minor to life-threatening, depending on how widespread the disease is and which parts of the body are affected.

The two main types of scleroderma are:
1) Localized scleroderma, which usually affects only the skin, although it can spread to the muscles, joints and bones. It does not affect other organs. Symptoms include discolored patches on the skin (a condition called morphea); or streaks or bands of thick, hard skin on the arms and legs (called linear scleroderma). When linear scleroderma occurs on the face and forehead, it is called en coup de sabre.
2) Systemic scleroderma, which is the most serious form of the disease, affects the skin, muscles, joints, blood vessels, lungs, kidneys, heart and other organs.
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.

What causes Scleroderma?

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.
Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.

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. Careful clinical evaluation is the primary method for monitoring scleroderma. X-rays and computerized tomography (CT) scans are used to look at bone abnormalities. Thermography can detect differences in skin temperature between the lesion and normal tissue. Ultrasound and magnetic resonance imaging (MRI) can aid soft tissue assessment. Some symptoms he or she will look for include:

1) 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. This is one of the earliest signs of the disease; more than 90 percent of patients with scleroderma have Raynaud's. Raynaud's can lead to finger swelling, color changes, numbness, pain, skin ulcers and gangrene on the fingers and toes. People with other diseases can also have Raynaud's and some people with Raynaud's do not have any other disease.
2) Skin thickening, swelling and tightening: This is the problem that leads to the name "scleroderma" ("Sclera" means hard and "derma" means skin). 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 (for example, fingers).
3) Enlarged red blood vessels on the hands, face and around nail beds (called "telangiectasias")
4) Calcium deposits in the skin or other areas
5) High blood pressure from kidney problems
6) Heartburn; this is an extremely common problem in scleroderma
7) Other problems of the digestive tract such as difficulty swallowing food, bloating and constipation, or problems absorbing food leading to weight loss
8) Shortness of breath
9) Joint pain