Scleroderma (systemic sclerosis [SSc]) is a chronic disease of unknown cause characterized by diffuse fibrosis of skin and visceral organs and vascular abnormalities.
Raynaud phenomenon is generally the presenting complaint (differentiated from Raynaud disease, generally affecting younger individuals and without digital ulcers).
Skin thickening, “pu...
Treatment is symptomatic and supportive.
Esophageal dilation may be used for strictures.
Avoid cold; dress appropriately in layers for the weather; be wary of air conditioning.
Monitor every 3 to 6 months for end-organ and skin involvement and medications. Provide encouragement.
Echocardiology and PFTs yearly
Herrick AL. Contemporary management of Raynaud’s phenomenon and digits ischaemic complications. Curr Opin Rheumatol. 2011;23(6):555–561. [View Abstract on OvidMedline]
M34.9 Systemic sclerosis, unspecified
M34.1 CR(E)ST syndrome
L94.0 Localized scleroderma [morphea]
L94.1 Linear scleroderma
M34.89 Other systemic sclerosis
M34.83 Systemic sclerosis with polyneu...
Raynaud phenomenon is frequently the initial complaint.
Skin thickening, “puffy hands,” and GERD are often noted early in disease.
Patients must be followed proactively for development o...
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