Six cases positive for anti-centromere antibodies with ulcer and gangrene in the extremities.

We report six cases that were positive for anti-centromere antibodies, with ulcer and gangrene in the extremities but mild or no skin thickening. The patients were five women and one man, and the mean age at onset of gangrene was 56 yr. Raynaud's phenomenon was found in five patients and calcinosis cutis in two patients. Three patients did not satisfy the criteria for systemic sclerosis and CREST syndrome in this study. Ulcer and gangrene occurred in the fingers in three patients, and in the fingers and toes in two patients. The gangrene was refractory to treatment, and amputation of fingers or toes was inevitable in five patients. Regardless of cutaneous lesion, the presence of anti-centromere antibodies may cause the same pathological presentation of vascular damage as seen in systemic sclerosis.