Hemiplegia and peripheral gangrene secondary to large and medium size vessels involvement in C.R.E.S.T. syndrome

A woman with CREST syndrome since the age of 35, had 11 and 13 years respectively after her disease onset, two episodes of CVA with residual right side hemiplegia. The angiography revealed segmented stenosis in the left common carotid, right subclavian and left renal arteries. At the age of 49 she developed gangrene of the right foot, requiring below the knee amputation. Pathological examination of the surgical specimen, showed extensive intimal fibrosis of the vessel walls in large and medium size arteries. Involvement of large and medium size arteries is infrequent in scleroderma. The case described illustrates this severe and unusual complication.