Scleroderma

Connective Tissue Disorder

Scleroderma Basics

Scleroderma is a connective tissue disorder that leads the immune system to mistakenly attack and destroy healthy body tissue. Some symptoms include:


  • hair loss
  • skin hardening or thickening
  • skin that is darker or lighter than normal
  • stiff or tight skin
  • small white lumps beneath the skin that ooze a toothpaste-like substance
  • sores on toes and fingers
  • joint pain, numbness, pain, stiffness, swelling in fingers and joints
  • dry cough, shortness of breath, wheezing
  • bloating, constipation, diarrhea, trouble swallowing, heartburn

Epidemiology and Pathogenesis

Scleroderma usually affects people 30-50 years old and is more common in women. It is unknown what causes the disease exactly; however, some people have a history of being exposed to silica dust and polyvinyl chloride.

Diagnosis and Management

A medical professional can identify this disorder through a physical exam (exam shows hard, thick skin) and checking your blood pressure (this disorder can lead to inflamed blood vessels in the kidney which leads to blood pressure problems). Other tests include chest x-rays, CT scan of the lungs, echocardiogram, urinalysis, and skin biopsy. Once the disorder is diagnosed, there are multiple routes of treatment. While there is no cure for Scleroderma, there are options including medication (Corticosteroids, drugs that suppress the immune system, and blood pressure medications) and light physical therapy.

Prognosis

In most cases, the disease will slowly get worse. Cases that only involve skin symptoms have a better outlook, but widespread scleroderma can lead to cancer, heart failure, scarring of lungs, high blood pressure in lungs, kidney failure, and problems absorbing nutrients. There are focus groups available and patients with this disorder usually find them helpful in dealing with scleroderma.