Differentials
Primary Raynaud's phenomenon
SIGNS / SYMPTOMS
Tends to have milder symptoms.
Attacks are symmetrical and precipitated by cold or emotional stress.
There is an absence of digital ulcers.
No history or physical signs suggestive of secondary cause.
INVESTIGATIONS
There are no diagnostic tests for primary Raynaud's phenomenon.
If done, the ANA is negative and capillaroscopy is normal.
Localised scleroderma (morphoea)
SIGNS / SYMPTOMS
Clinical and histopathological skin changes that are indistinguishable from systemic sclerosis.
May be in a linear distribution (linear scleroderma).
Most often seen in children and young adults, rather than older adults.
There is a lack of Raynaud's phenomenon, sclerodactyly, or visceral manifestations.
INVESTIGATIONS
Abnormal chest x-ray, pulmonary function tests, barium swallow make the diagnosis of scleroderma more likely than localised scleroderma (morphoea).
Diagnosis is clinical.
Mixed connective tissue disease
SIGNS / SYMPTOMS
Patients typically have features of systemic lupus erythematosus, scleroderma, and polymyositis.
INVESTIGATIONS
May have testing consistent with features of scleroderma.
High-titre anti-RNP antibodies usually present.
Eosinophilic fasciitis
SIGNS / SYMPTOMS
Associated with over-exertion.
Leads to adherence of skin to underlying fascia, usually sparing the hands and feet.
Raynaud's phenomenon is absent.
INVESTIGATIONS
Peripheral eosinophilia.
Chest x-ray shows no evidence of interstitial lung disease.
Pulmonary function tests (PFTs) show no evidence of interstitial lung disease or pulmonary artery hypertension associated with scleroderma (PFTs indicate pulmonary hypertension if the diffusing capacity of the lung for carbon monoxide is disproportionately decreased in comparison with the forced vital capacity).
Negative ANA.
Deep biopsy, including the fascia, is important to make the diagnosis. It shows an inflammatory infiltrate with eosinophils.
Scleromyxoedema
SIGNS / SYMPTOMS
Characterised by waxy, yellow-red papules on the head, neck, arms, and upper trunk, occurring over thickened and indurated skin.
Associated with a diagnosis of amyloidosis.
INVESTIGATIONS
Monoclonal spike on serum or urine protein electrophoresis.
Chest x-ray shows no evidence of interstitial lung disease.
Pulmonary function tests (PFTs) show no evidence of interstitial lung disease or pulmonary artery hypertension associated with scleroderma (PFTs indicate pulmonary hypertension if the diffusing capacity of the lung for carbon monoxide is disproportionately decreased in comparison with the forced vital capacity).
Sclerodema (scleroderma diabeticorum and sclerodema of Buschke)
SIGNS / SYMPTOMS
Most patients also have diabetes.
Characterised by skin thickening, affecting the neck, upper back, shoulders, and trunk.
Raynaud's phenomenon and internal organ involvement absent.
INVESTIGATIONS
Chest x-ray shows no evidence of interstitial lung disease.
Pulmonary function tests (PFTs) show no evidence of interstitial lung disease or pulmonary artery hypertension associated with scleroderma (PFTs indicate pulmonary hypertension if the diffusing capacity of the lung for carbon monoxide is disproportionately decreased in comparison with the forced vital capacity).
Negative ANA and anti-topoisomerase I antibody.
Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy
SIGNS / SYMPTOMS
Occurs in patient with renal compromise, usually end-stage renal disease. Associated with gadolinium administration.
Skin changes may be indistinguishable from scleroderma but Raynaud's phenomenon is usually absent.
May have internal organ fibrosis.
INVESTIGATIONS
Chest x-ray shows no evidence of interstitial lung disease.
Pulmonary function tests (PFTs) show no evidence of interstitial lung disease or pulmonary artery hypertension associated with scleroderma. PFTs indicate pulmonary hypertension if the diffusing capacity of the lung for carbon monoxide is disproportionately decreased in comparison with the forced vital capacity.
Negative ANA and anti-topoisomerase I antibody. Negative anti-RNA polymerase III antibody (there is no correlation with nephrogenic systemic fibrosis and the presence of this auto-antibody).
Skin biopsy has distinct pathological findings that distinguish it from scleroderma.
Acrodermatitis chronicum atrophicans
SIGNS / SYMPTOMS
Associated with Borrelia infection.
Has a chronically progressive course leading to skin atrophy.
Favours the lower extremities and is generally symmetric.
INVESTIGATIONS
Responds to antibiotics.
Chest x-ray shows no evidence of interstitial lung disease.
Pulmonary function tests (PFTs) show no evidence of interstitial lung disease or pulmonary artery hypertension associated with scleroderma (PFTs indicate pulmonary hypertension if the DLCO is disproportionately decreased in comparison with the FVC).
Negative ANA and anti-topoisomerase I antibody.
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