Limited cutaneous systemic sclerosis
Limited Cutaneous Systemic Sclerosis (lcSSc) – Understanding the Condition
What is Limited Cutaneous Systemic Sclerosis?
Limited cutaneous systemic sclerosis (lcSSc) is a subtype of systemic sclerosis (scleroderma), an autoimmune connective tissue disease that causes fibrosis (hardening) of the skin and internal organs. In lcSSc, skin thickening is typically restricted to the hands, forearms, face, and lower legs, distinguishing it from diffuse systemic sclerosis, which affects larger areas of the body.
This condition was historically referred to as CREST syndrome, an acronym representing its key features:
- Calcinosis – Calcium deposits in the skin and tissues
- Raynaud’s phenomenon – Reduced blood flow causing fingers/toes to turn white or blue in cold temperatures
- Esophageal dysmotility – Difficulty swallowing due to reduced esophageal movement
- Sclerodactyly – Thickened, tight skin on the fingers
- Telangiectasia – Dilated blood vessels appearing as red spots on the skin
Causes and Risk Factors
The exact cause of lcSSc remains unknown, but it is believed to result from a dysregulated immune system that triggers excessive collagen production. Risk factors include:
- Genetic predisposition
- Environmental triggers (e.g., silica exposure)
- Female sex (more common in women)
Symptoms of lcSSc
- Skin tightening on the fingers, hands, face, and forearms
- Raynaud’s phenomenon – A common early symptom
- Joint pain and stiffness
- Difficulty swallowing and acid reflux (due to esophageal involvement)
- Telangiectasia (tiny red blood vessel lesions on skin)
- Pulmonary hypertension (in some cases, affecting lung blood vessels)
Complications
While lcSSc progresses more slowly than diffuse systemic sclerosis, it can still lead to complications, such as:
- Pulmonary arterial hypertension (PAH) – A serious condition affecting blood flow to the lungs
- Gastrointestinal issues – Acid reflux, slowed digestion
- Calcinosis – Calcium deposits under the skin, causing pain and infection risk
Diagnosis and Testing
Diagnosis is based on clinical symptoms and antibody testing, including:
- Anti-Centromere Antibodies (ACA) – Found in ~70-80% of lcSSc cases
- Antinuclear Antibody (ANA) Panel
- Pulmonary function tests and echocardiogram (to assess lung and heart involvement)
Treatment and Management
There is no cure, but treatment focuses on managing symptoms and preventing complications:
- Medications for Raynaud’s phenomenon (calcium channel blockers, vasodilators)
- Proton pump inhibitors for acid reflux
- Immunosuppressants for severe cases
- Physical therapy to maintain mobility
Key Takeaways
- Limited cutaneous systemic sclerosis affects mainly the skin of the hands, face, and forearms but can impact internal organs.
- It progresses more slowly than diffuse scleroderma but can lead to pulmonary hypertension and digestive issues.
- Early detection and management can improve quality of life.
If you suspect lcSSc or have a positive Anti-Centromere Antibody (ACA) test, consult a rheumatologist for further evaluation and monitoring.
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Biomarkers related to this condition:
The Anti-Centromere Antibody (ACA) test is an indirect immunofluorescence assay (IFA) used to detect autoantibodies that target centromere proteins. These antibodies are primarily associated with autoimmune connective tissue diseases, particularly li
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