A11. Scleroderma and its variants

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