Scleroderma

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Scleroderma Renal Crisis

Scleroderma

Systemic sclerosis

• multisystem disease of unknown cause
• can be localised to skin +/- internal organ involvement

Types

• morphoea
  • localised patch of skin involvement
• limited cutaneous scleroderma
  • affects limited to skin
  •  

Pathology

• vascular lesions
  • involving
    • small arteries
    • arterioles
    • capillaries
  • endothelial damage
  • vasoconstriction
  • intimal damage leading to increased vascular permeability
  • migration of cells into extracellular matrix
  •  widespread obliterative arterial lesions
  • chronic ischaemia
• fibrosis
  • fibroblasts synthesise collagen I+II, fibronectin and glycosaminoglycans
  • produces fibrosis in lower dermis + internal organs

Examination

• Introduction, exposure
• Observation
  • General
    • signs of Mixed Connective Tissue Disease
    • battery heated gloves (cold hands)
  • Face
    • tight skin around mouth
    • telangiectasia
    • dry eyes
• Hands
  • Skin
    • tight
    • shiny
    • spindle shaped fingers
    • calcinosis
    • Raynaud's Phenomenon
    • Pulp infarcts
    • autoamputation
  • Nails
    • nail bed infarcts
  • Joints
    • swollen
  • Nerves
    • usually none
    • Carpal tunnel syndrome
    • Proximal muscle weakness
  • Function
    • may be severely restricted
• Arms
  • skin changes over much of body surface

Complications

• Renal Crisis

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