Scleroderma
Key points
- Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.
- The disease may produce local or systemic symptoms.
- Signs and tests Examination of the skin may show tightness, thickening, and hardening.
- In the majority of those affected, the disease is progressive.
Alternative names
CREST syndrome; Progressive systemic sclerosis; Systemic sclerosis
Definition
Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.
Causes, incidence, and risk factors
The cause of scleroderma is unknown. The disease may produce local or systemic symptoms. The course and severity of the disease varies widely in those affected.
Excess collagen deposits in the skin and other organs produce the symptoms. Damage to small blood vessels within the skin and affected organs also occurs. In the skin, ulceration, calcification, and changes in pigmentation may occur.
Systemic features may include fibrosis and degeneration of the heart, lungs, kidneys and gastrointestinal tract.
The disease usually affects people 30 to 50 years old. Women are affected more often than men. Risk factors are occupational exposure to silica dust and polyvinyl chloride.
Symptoms
- blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
- pain, stiffness, and swelling of fingers and joints
- skin thickening and shiny hands and forearm
- skin is hard
- tight and mask-like facial skin
- ulcerations on fingertips or toes
- esophageal reflux or heartburn
- difficulty swallowing
- bloating after meals
- weight loss
- diarrhea
- constipation
- shortness of breath
Additional symptoms that may be associated with this disease:
- wrist pain
- wheezing
- skin, abnormally dark or light
- joint pain
- hair loss
- eye burning, itching and discharge
Signs and tests
Examination of the skin may show tightness, thickening, and hardening.
- The ESR is elevated.
- The rheumatoid factor can be elevated.
Frequently asked questions
What is scleroderma?
Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.
What are the risk factors for scleroderma?
The cause of scleroderma is unknown. The disease may produce local or systemic symptoms. The course and severity of the disease varies widely in those affected. Excess collagen deposits in the skin and other organs produce the symptoms. Damage to small blood vessels within the skin and affected organs also occurs. In the skin, ulceration, calcification, and changes in pigmentation may occur. Systemic features may include fibrosis and degeneration of the heart, lungs, kidneys and gastrointestinal tract. The disease usually affects people 30 to 50 years old. Women are affected more often than men. Risk factors are occupational exposure to silica dust and polyvinyl chloride.
What are the symptoms of scleroderma?
- blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
- pain, stiffness, and swelling of fingers and joints
- skin thickening and shiny hands and forearm
- skin is hard
- tight and mask-like facial skin
- ulcerations on fingertips or toes
- esophageal reflux or heartburn
- difficulty swallowing
- bloating after meals
- weight loss
- diarrhea
- constipation
- shortness of breath Additional symptoms that may be associated with this disease:
Written and revised by
David Cohen, M.D.
Our doctors write and review articles for accuracy and keep them up to date.
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