Scleroderma, also known as Systemic Sclerosis(SS), is a chronic connective tissue disease generally classified as one of the Autoimmune Rheumatic diseases. The word “Scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin, therefore hardening of the skin is one of the most visible manifestations of this disease.
Scleroderma or (SS) often only affects the skin early in the stages of the disease. You may notice a thickening of your skin, and shiny areas developing around your mouth, nose, fingers, and other bony areas.
As the condition progresses, you may begin start to have limited movement of the affected areas. Other symptoms may include the following:
- hair loss
- calcium deposits, or white lumps under the skin
- small, dilated blood vessels under the skin’s surface
- joint pain
- shortness of breath
- a dry cough
- difficulty swallowing
- esophageal reflux
- abdominal bloating after meals
You may also begin to experience spasms of the blood vessels in your fingers and toes. Then, your extremities may turn white and blue when you’re in the cold or feeling extreme emotional stress. This is called Raynaud’s phenomenon.
The disorder isn’t confined to skin changes. It can also affect your:
- blood vessels
- digestive system
- Scleroderma is not contagious
Some features of systemic sclerosis can appear in other autoimmune disorders. When this occurs, it’s called a mixed connective disorder.
The disease is typically seen in people 30 to 50 years old, but it can be diagnosed at any age. Women are more likely than men to be diagnosed with this condition. The symptoms and severity of the condition vary from one person to another based on the systems and organs involved.
Systemic Sclerosis is also called Scleroderma, progressive systemic sclerosis, or CREST syndrome. “CREST” stands for:
- Raynaud’s phenomenon
- esophageal dysmotility
CREST syndrome is a limited form of the disorder.
Scleroderma occurs when your body begins to overproduce collagen and it accumulates in your tissues. Collagen is the main structural protein that makes up all of your tissues.
Doctors aren’t sure what causes the body to produce too much collagen. The exact cause of Scleroderma is currently unknown.
There are some risk factors that can make you more likely to contract Scleroderma (SS)
- being Native American
- being African-American
- being female
- using certain chemotherapy drugs such as Bleomycin
- being exposed to silica dust and organic solvents
It is important to see a Rheumatologist and get a diagnosis. Diagnostic tests can include the following:
- a chest X-ray
- a urinalysis
- a CT scan of the lungs
- skin biopsies
Treatment can’t cure the condition, but it can help reduce symptoms and slow disease progression. Treatment will typically be decided based on a person’s symptoms and the need to prevent complications.
Treatment for generalized symptoms may involve:
- immunosuppressants, such as methotrexate or Cytoxan
- nonsteroidal anti-inflammatory drugs
Depending on your symptoms, treatment can also include:
- blood pressure medication
- medication to aid breathing
- physical therapy
- light therapy, such as ultraviolet or A1 phototherapy
- nitroglycerin ointment to treat localized areas of tightening of the skin
What to Expect
You can make lifestyle changes to stay healthy with Scleroderma, such as avoiding smoking cigarettes, remaining physically active, and avoiding foods that could trigger heartburn.
Treatments for Scleroderma have drastically improved in the past 30 years. Although there’s still no cure for SS, there are several different treatments that could help you manage your symptoms. Talk to your doctor if any of your symptoms are getting in the way of your daily routine. They can work with you to adjust your treatment plan and improve your daily lifestyle.
Your Doctor may know of support groups for people with your condition. Sometimes talking with others also coping with the same condition can help. Ask your Doctor to help you find a support group.