The Differential Diagnosis of Systemic Sclerosis

Alan Tyndall; Susanna Fistarol


Curr Opin Rheumatol. 2013;25(6):692-699. 

In This Article

Local Mimics of Systemic Sclerosis

Although not diffuse and symmetrical, several local skin thickening syndromes may present as suspected SSc. These include morphea, also called localized scleroderma, with its five subtypes: plaque-type morphea, bullous morphea, linear morphea, generalized morphea and deep morphea. A particular form of linear scleroderma is scleroderma en coup de sabre. The distinction between linear scleroderma en coup de sabre and progressive facial hemiatrophy (Parry–Romberg syndrome) can be difficult and their relationship is unclear. Stiff skin syndrome is another scleroderma-like localized disorder. The absence of typical SSc-associated features, for example, specific autoantibodies, vascular instability, nailfold capillaroscopy changes and other organ involvement and lack of symmetry help to exclude SSc.