Cutaneous features
- Raynaud’s phenomenon
- ‘Hide-bound’ skin
- Classical sclerodermoid facies- “mask-like” face, pinched or beak-like appearance of nose, radial furrows around the mouth and thinning of the upper lip
- Pigmentation – mottled or hyperpigmentation
- Swelling of hands & joints, atrophy
- Finger and leg ulcers, digital gangrene, stellate scars
- Nail fold telangiectasias
- Calcinosis
Systemic Features
- Gastrointestinal and Hepatic - Esophageal Dysfunction, malabsorption, primary biliary cirrhosis, Autoimmune hepatitis.
- Arthritis, tendon friction rubs.
- Renal-scleroderma renal crisis (most severe).
- Lung-Interstitial lung disease, Pulmonary hyertension.
- Cardiac-myocardial disease, pericardial involvement.
- Muscle-SSc Associated myopathy more common in diffuse SSc.
- Digital infarctions
- Pulmonary hypertension
- Myositis
- Renal failure
- Wound infections
- Immune activation, fibrosis of skin and obliterative vasculopathy.
- Females : Males - 5.2 : 1
- Onset : Fourth decade
