Systemic connective tissue disease causing fibrosis and vascular damage to the skin and internal organs

Excessive collagen deposition as a result immunologic system disturbances and vascular changes

Associated with malignancy

Affected organs and systems include the skin, lungs, heart, digestive system, kidneys, muscles, joints, and nervous system

2 forms:

Limited Cutaneous: Systemic symptoms occur late, skin of extremities 

affected early. 

Diffuse Cutaneous: Rapidly progressing, early systemic symptoms. 

Limited Cutaneous: CREST Syndrome

Calcinosis

Raynaud’s syndrome 

Esophageal dysmotility

Sclerodactyly

Telangiectasia  

Diffuse Cutaneous: Rash and thickened skin in multiple areas

Pruritus, Raynaud’s phenomenon, dysphagia, nausea, vomiting, weight loss, abdominal cramps, bloating, diarrhoea, shortness of breath, dry cough, palpitations, arthralgia, myalgia, weakness.

Bloods: FBC shows anaemia, Raised ESR, Raised creatinine kinase.

Auto-antibodies: Scl-70 associated with diffuse type. Anti-centromere, ANA

Imaging: Chest X-Ray to assess for presence of lung fibrosis

Echo to assess for cardiac involvement

Lung function tests to assess lung volumes and disease progression

Medical: Topical emollients, calcium-channel blockers, vasodilating drugs,

ACE inhibitors, PPI, corticosteroids

Poor prognosis with No cure. 

Smptomatic management 

Pulmonary hypertension can be life threatening