Scleroderma

Scleroderma is a disease characterized by an overproduction of collagen due to immune dysregulation. This can lead to a hardening of the skin and, in some cases, of internal organs such as the lungs, blood vessels, heart, kidneys, and digestive system. Hardening of the skin can limit motor skills and dexterity, and the affected organs may not function normally. Scleroderma is not contagious.

There are two main forms of the disease:

• The localized form, known as morphea, which affects only the skin
• The systemic form, which affects the skin and internal organs

Systemic scleroderma, in turn, is classified into two types:

• Limited scleroderma, or CREST syndrome, affects the skin on the extremities (hands, forearms, feet, below the knees, face, and neck)
• Diffuse scleroderma affects a larger area of the skin

Causes and triggers

Scleroderma is an autoimmune disorder. This type of illness occurs when your immune system attacks your own cells. The exact cause remains unknown. Triggers can include exposure to a virus or a chemical substance.

Scleroderma can develop at any age but most often appears when individuals are in their 40s. It is 5 to 6 times more common in women than in men. In addition, individuals with a family history of scleroderma may be at increased risk of related diseases (e.g., rheumatoid arthritis).

Complications may initially go unnoticed:

• Decreased circulation (pallor or numbness) in the fingers or toes in response to cold or stress, a symptom called Raynaud's phenomenon
• Gastroesophageal reflux (burning sensation that rises in the throat)
• Shortness of breath with physical exertion, dry cough

Whitening or numbness of the fingers or toes (Raynaud's phenomenon) is often the first sign of scleroderma, preceding the appearance of other symptoms by several years.

Treatment

There is as yet no cure for scleroderma. However, most of the disease's complications can be treated.

When should I see a health care professional?

Speak with your health care provider in the following situations:

• You develop numbness or pallor in your fingers or toes, or these symptoms become more frequent or severe (Raynaud's phenomenon)
• You have a slow-healing wound on the tip of a finger or toe, or a wound exuding discharge
• You feel like there's something blocking your esophagus when you swallow
• You feel more out of breath or dizzier than usual during regular activities (e.g., cleaning)
• You have swollen legs
• You have persistently high blood pressure

For more information:

• Scleroderma Quebec