Care and mobility considerations
Disabling effects of Scleroderma
The disabling effects of scleroderma vary widely depending on the type of disease and its manifestations. The main disabling effects are due to limb involvement, particularly contractures and ulceration of digits. Later in the course of systemic disease, significant effort tolerance limitation may occur due to pulmonary hypertension.
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Condition
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Manifestations
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Disabling Effects
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Localised Scleroderma
a.) Localised Morphea
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- One to several patches of scleroderma (thickened skin)
- Affects only the skin and subcutaneous fat
- Can occur all over the body, but usually affects trunk
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- Effects are cosmetic (psychological) – there are no care or mobility needs.
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Localised Scleroderma
b.) Generalised Morphoea
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- Less common
- More severe
- Large patches thick tight skin affecting trunk, arms and legs.
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- Rarely, if the morphea is extensive, respiration may be affected (therefore exercise tolerance and mobility)
- If there is joint involvement, there may be contractures which would reduce mobility (lower limbs) and use of upper limb(s)
- Carpal tunnel syndrome may occur if wrist area affected
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Localised Scleroderma
c.) Linear Scleroderma
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- Usually presents in childhood (<14 years)
- Highly visible bands of thick tight skin
- Follows distribution of skin dermatomes and can damage structures down to bones and joints.
- “En coup de sabre” deformity, patients may have seizures, headaches, trigeminal neuralgia, muscle weakness, hemiparesis and visual changes.
- Both sides often affected
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- Occurrence in children often leads to severely affected limb growth.
- Disabling effects would depend on the extent and type of lesion
- Peripheral nerve involvement may occur
- Mobility may be restricted
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Systemic Scleroderma
a.) Limited Cutaneous Systemic Sclerosis (Crest Syndrome)
Accounts for at least 60% of cases
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- Long history of Raynaud’s Phenomenon (see below)
- Skin changes involve hands, face, feet and forearms
- Also known as CREST
- High incidence of pulmonary arterial hypertension arising around 10 years following diagnosis
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- Raynaud’s phenomenon can affect manual dexterity. Further complications such as ulceration of extremities, loss of tips of fingers, contractures and sclerodactyly also impair manual dexterity, and therefore may affect many aspects of self-care and meal preparation. Additionally, coping with bowel incontinence if present, may prove difficult with dexterity problems.
- Fatigue is a major symptom that can limit quality of life.
- Pulmonary arterial hypertension may affect exercise tolerance and therefore reduce mobility.
There may be difficulties with eating because of small, tight mouth, lack of saliva, loose teeth and swallowing problems.
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- Rapid onset
- Diffuse swelling and stiffness of fingers leads to sclerosis followed by involvement of the trunk. Raynaud’s disease may develop later
- Systemic features (kidneys, lungs, heart, and gastrointestinal system)
- Skin disease rapidly progresses
- Joint, muscle and bone involvement
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- As in the limited cutaneous form, manual dexterity can be affected (see above for effects)
- Joint involvement may lead to contractures which would reduce mobility (lower limbs) and use of the upper limbs
- Fatigue, kidney failure, pulmonary hypertension, cardiac involvement may all lead to reduced exercise tolerance, and subsequent care and mobility needs.
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- No skin changes
- May have Raynaud’s Phenomenon
- Complications of lungs, heart, kidneys or gastrointestinal system.
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