Direct immunofluorescent findings in scleroderma syndromes

Cutaneous direct immunofluorescent findings were examined in 78 patients who had either vascular scleroderma (group 1, 52 patients) or scleroderma with features of myositis or lupus erythematosus (group 2, 26 patients). Group 2 had higher antinuclear antibody levels, erythrocyte sedimentation rates, serum IgG concentrations, frequency of positive LE clot test, and rheumatoid factor activity. Ninety-two percent of group 1 (48 patients) had negative direct immunofluorescent findings, whereas 77% of group 2 (20 patients) had positive findings at the basement membrane or in the blood vessels (or both). The 6 patients in group 2 who had negative immunofluorescent findings were all on systemic steroid therapy. Of the 17 patients in group 2 who had tests for antibody to extractable nuclear antigen, only 3 had high-titer antibody to ribonucleoprotein--a pattern characteristic of mixed connective tissue disease. Direct cutaneous immunofluorescence is proposed as a means of identifying those patients with scleroderma who may be steroid-responsive.