Clinical features

1) Raynaud’s Phenomenon

Sudden, reversible colour change of extremities; may precede onset of scleroderma
Virtually universal in scleroderma
Reduction of the blood supply to the digits, poor healing, and in extreme cases can lead to ulceration, gangrene and loss of the tips of the digits
Usually affects fingers and toes, but may also affect nose and ear and other extremities

Bands or patches of thickened skin in various parts of the body, depending on the type of disease
Salt-and-pepper skin discoloration (areas of hyper-pigmentation alternating with hypo-pigmentation)
Early phase of disease, fingers may be swollen and sausage shaped
Sclerodactyly occurs later, skin of fingers tight and shiny with associated loss of normal skin creases, fingers tightened into a bent “claw” position.
Calcium deposits under the skin (“calcinosis”).
Thickened and tight skin causes puckering and wrinkling around the mouth with thinning of the lips and small mouth (“microstomia”). This often makes dental care difficult
“Beaking” of the nose.
Telangectasia – small red spots in the skin associated with swelling of small blood vessels.

Characteristic sclerodactyly with tight, shiny skin, ·clawing· of fingers; ulceration of fingers and discoloration of skin

Muscle weakness and wasting can occur because of associated myositis (inflammation of the muscles).

Most frequent cause of death
Fibrosing alveolitis (diffuse disease of the lung tissue). Lung fibrosis can impair gas exchange, leading to exertional dyspnoea and restrictive disease with eventual respiratory failure.
Pulmonary arterial hypertension often develops later in systemic disease

Acute hypertensive renal crisis has become rare as treatment has improved. The structural vascular changes and possibly vasospasm (renal Raynaud’s phenomenon), caused by scleroderma can lead to decreased blood flow to the kidney thus precipitating renal failure. Renal crisis is also associated with the use of corticosteroids. Urinary urgency and frequency due to bladder wall changes
Women may experience vaginal dryness, dyspareunia (painful intercourse), and menstrual irregularities.
Erectile dysfunction likely due to fibrosis of erectile tissue

Scleroderma is a difficult condition to treat, manage and live with due to its complex effects.
Psychosocial and functional problems often arise from fatigue, pain, sexual dysfunction and disfigurement.
The visible skin changes often cause patients to have a negative body image leading to depression.
Bowel incontinence is more common than previously thought and is particularly distressing. It is often under-reported and treatment options are limited causing patients to stay at home leading to isolation.
It often restricts the social activities and quality of life of its sufferers.
Living with a chronic disease that is incurable and has a high mortality and morbidity can cause a significant amount of psychological distress.
Support from specialist nurses and mental health professionals are important in enabling these patients to cope with the disease.
The exchange of experience and counselling has proven to be very helpful in self-help groups.
Those with end-stage disease require provision of access to end-of-life specialised palliative treatment and psychological support.

Amended November 2008