Scleroderma

From Ganfyd

Scleroderma (dermatosclerosis) is a term used for a number of conditions in which there is an abnormal overgrowth of connective tissue.

Scleroderma may be divided into three patterns:

• Localised (morphea) - affecting the skin predominantly
• Limited ie CREST syndrome - calcinosis, Raynaud's syndrome, (o)esophageal involvement, (distal) sclerosis, telangiectasia
• Systemic - affecting other organs in addition to the skin, so better name is systemic sclerosis

Symptoms

• Thickening and tightening of skin on hands and face, particularly the fingers.
• Pale skin
• Raynaud's phenomenon
• Splits and ulcers in the finger pulps
• Small abscesses may form around the nails

Symptoms may initially develop in one hand or even one finger, gradually moving across to involve the other.

Systemic disease presents with symptoms of the involved organs eg dysphagia, constipation or colicky abdominal pain in gastointestinal involvement.

Signs

• Skin becomes thickened, white, waxy (although Morphea can have different skin appearances)
• Characteristic bird-like facies with botox-like forehead and microstomia
• Spindly fingers
• Cold hands, +/- colour changes (Raynaud's)
• Calcinosis - particularly a feature of CREST syndrome
• Decreased movement of the interphalangeal joints of the hand (due to thickened skin)
• Telangiectasia (in CREST) particularly at nail beds and gums. Capillaroscopy helpful
• Myositis
• Dry synovitis (ie with little inflammation)

Systemic

• GI - strictures
• Cardiovascular - hypertension, proteinuria, arrhythmias
• Pulmonary fibrosis

Diagnosis

Clinically features are characteristic. Most have some auto-antibodies:

• Anti-Centromere antibodies seen in CREST
• Extractable nuclear antibodies (ENA) esp anti-Scl-70 seen in other forms

Calcinosis may be apparent on X-ray. Deep tissue involvement should be looked for with MRI if extensive morphea.

Management

Screen with ECG and lung function. Otherwise symptomatic eg

• Pamidronate for calcinosis

Prognosis

Variable, progression can be erratic.