Scleroderma

Scleroderma, or systemic sclerosis, is a chronic autoimmune disease primarily affecting the skin and connective tissues but can also involve internal organs such as the lungs, heart, and kidneys.

Cause

The exact cause is unknown, but it is believed to result from a combination of genetic predisposition and environmental factors that trigger the immune system to produce excess collagen, leading to fibrosis.

Types

• Limited Cutaneous Scleroderma (formerly known as CREST syndrome):
  • Limited skin involvement, affecting the face, hands, and forearms.
  • Calcium deposits (Calcinosis).
  • Raynaud’s phenomenon:
    Fingers turning colors in response to cold or stress.
  • Esophageal dysfunction.
  • Telangiectasias:
    Dilated blood vessels under the skin.
• Diffuse Cutaneous Scleroderma
  • Widespread skin involvement, including the trunk, arms, and legs.
  • Rapid progression that can affect the lungs, heart, kidneys, and gastrointestinal tract.

Symptoms

• Skin tightening, hardening, and reduced flexibility.
• Ulcers or sores on the fingertips.
• Raynaud’s phenomenon.
• Acid reflux, difficulty swallowing, bloating, diarrhea, or constipation.
• Weight loss or malnutrition in severe cases.
• Shortness of breath.
• Joint pain, swelling, or muscle weakness.
• Potential kidney problems.

Treatment

• There is no cure for scleroderma, but treatment aims to manage symptoms, slow disease progression, and prevent complications.
• Medications include immunosuppressants, vasodilators to improve blood flow, acid reflux medications, and pain relievers.
• Physical therapy and occupational therapy.
• Skin care, including regular moisturizing.
• Regular monitoring and treatment of organ involvement.
• Collaboration with multiple specialists, including rheumatologists, cardiologists, and pulmonologists.