Scleroderma

From greek.doctor
  • Scleroderma = Abnormal growth of connective tissue
  • In all types the skin is thickened and hardened
  • Classification of scleroderma
    • Systemic sclerosis/systemic scleroderma
      • Limited systemic sclerosis/CREST syndrome
      • Diffuse systemic sclerosis
      • Systemic sclerosis sine scleroderma
    • Localized scleroderma
      • Morphea
      • Linear scleroderma
  • Systemic sclerosis
    • Sclerosis of internal organs in addition to the skin
    • Skin symptoms
      • Thickening and hardening of skin
      • Depigmentation (except perifollicular area)
        • Salt-and-pepper appearance
      • Sclerodactyly
      • Face
        • Loss of expression
        • Shortened frenulum
        • Microstomia
    • Limited systemic sclerosis/CREST syndrome
      • More common
      • Skin manifestations are limited to hands, fingers and face
      • Organ involvement is rare and limited
      • Clinical features
        • Calcinosis cutis – calcium deposits on pressure points of extremities
        • Raynaud’s phenomenon
        • Esophageal hypomobility
        • Sclerodactyly
          • Red-blue discoloration of fingers
          • Fibrosis of fingers
          • Atrophy and necrotic spots
        • Telangiectasia
    • Diffuse systemic sclerosis
      • Less common
      • More aggressive
      • Early organ involvement
      • Clinical features
        • Oesophageal dysmotility
        • Small bowel dysmotility
        • Pulmonary hypertension
        • Interstitial lung disease
        • Cardiac fibrosis
        • Renal disease
  • Localized scleroderma
    • No organ involvement, only skin
    • Only localized parts of the skin are affected
    • Morphea
      • Isolated patches of hardened skin
      • No organ involvement
      • Treatment
        • PUVA
        • Intralesional steroid injection
    • Linear scleroderma
      • Starts as a line of hardened skin
      • Develops in childhood
      • Treatment
        • PUVA