Scleroderma is a disorder marked by hardening of the body’s connective tissue, but can also involve internal organs such as the heart, lungs, kidneys and the gastrointestinal tract.
Symptoms vary greatly from person to person.
These are some of the most frequent signs of scleroderma:
- Swelling of the fingers and hands, which progresses into thickening of the skin
- Tautness and hardening of the skin of the hands, which may spread to the forearms, trunk, face and feet
- Hard patches on the skin, usually white in color
- Lines of thickened skin on the arms, legs and forehead
- Hair loss
- Raynaud’s syndrome, a condition in which the fingers and toes become white and blue during exposure to cold
- Heartburn and indigestion
Eventually, scleroderma may progress to affect the internal organs of the body, a condition called progressive systemic sclerosis.When the lungs are affected, tissue is thickened, interfering with normal passage of oxygen, which leads to breathing difficulties.The heart is rarely affected, but when it is, fibrotic tissue develops in the heart muscle, leading to compromised pumping and, possibly, heart failure.Kidney involvement is usually marked by a sudden spike in blood pressure, followed by a rapid kidney failure.
Kidney failure is the most frequent cause of death in patients with scleroderma.Scleroderma varies greatly in terms of severity. In some patients, it only involves the fingers and the toes, resulting in nothing more than sensitivity to cold. Scleroderma’s progression is hard to predict. It can remain a fairly mild disorder for years, and only then evolve into a more serious disorder.
Some people develop a form of scleroderma called CREST, marked by tight skin, esophageal dysfunction, spider-like veins on the skin surface and Raynaud’s syndrome. This form of scleroderma rarely progresses to the more severe form of the disease, which involves internal organs.
Who Gets Scleroderma?
Although scleroderma is more common in women, it can also occur in men and children. It affects people of all races and ethnic groups. However, there are some patterns by disease type:
- Localized forms of scleroderma are more common in people of European descent than in African Americans.
- Morphea usually appears between the ages of 20 and 40.
- Linear scleroderma usually occurs in children or teenagers.
- Systemic scleroderma, whether limited or diffuse, typically occurs in people from 30 to 50 years old. It affects more women of African American than European descent.
The National Institute for Arthritis and Muscloskeletal and Skin Diseases (NIAMS) estimates the number of people in the United States with systemic sclerosis to be anywhere between 40,000 and 165,000. The number of people affected by all forms of scleroderma and scleroderma-like conditions ranges anywhere between 250,000 and 992,500, the NIAMS estimates.
Scleroderma is believed to be an auto-immune disorder, but the exact cause of the disease remains a mystery. Researchers have found that people with scleroderma produce too much collagen, a protein that builds up and causes thickening and hardening of the skin and, sometimes, of the internal organs.
Scleroderma is diagnosed by performing a medical exam and running some lab tests or taking a biopsy.
There is no cure for slceroderma, and treatment is limited. Certain drugs used in rheumatoid arthritis have shown promise in curbing the progression of scleroderma.