Scleroderma (systemic sclerosis [SSc]) is a chronic disease of unknown etiology, characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, articular structures and other organs (kidneys, lung, heart, gastrointestinal and skeletal muscles). The majority of manifestations have vascular features (e.g., Raynaud's phenomenon), but frank vasculitis is rarely seen. It can range from a mild disease, affecting the skin, to a systemic disease that can cause death in a few months.

• Divided into two major clinical variants:
  • Diffuse - distal and maximal extremity and truncal skin thickening
  • Limited - restricted to the fingers, hands and face. CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmobility, sclerodactyly, telangiectasia) closely analogous with limited scleroderma.
• System(s) affected: Skin/Exocrine, Renal/Urologic, Cardiovascular, Gastrointestinal, Musculoskeletal, Pulmonary
• Genetics: Familial clustering is rare, but has been seen
• Incidence/Prevalence in USA: 1/100,000
• Predominant age:
  • Young adult (16–40 years); middle age (40–75 years)
  • Symptoms usually appear in the 3rd to 5th decade
• Predominant sex: Female > Male (4:1)

• Skin
  • Digital ulcerations
  • Tightness, swelling, thickening of digits
  • Hyperpigmentation, hypopigmentation
  • Narrowed oral aperture
  • Pruritus
  • Scaling of skin
  • Subcutaneous calcinosis
• Peripheral vascular system
  • Raynaud's phenomenon
  • Telangiectasia
• Joints, tendons and bones
  • Flexion contractures
  • Friction rub on tendon movement
  • Hand swelling
  • Joint stiffness
  • Polyarthralgia
  • Sclerodactyly
• Muscle
  • Proximal muscle weakness
  • Weakness
• Gastrointestinal tract
  • Dysphagia
  • Esophageal reflux
  • Malabsorptive diarrhea
  • Nausea and vomiting
  • Weight loss
  • Xerostomia
• Kidney
  • Hypertension
• Pulmonary
  • Dry crackles at lung bases
  • Dyspnea
• Nervous system
  • Peripheral neuropathy
  • Trigeminal neuropathy

• Unknown
• Possible alterations in immune response
• Possibly some association with quartz mining, quarrying, vinyl chloride, hydrocarbons, toxin exposure, rape seed oil
• Treatment with bleomycin has caused a scleroderma-like syndrome

Unknown