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Background  We previously demonstrated that fasciitis is a common lesion in dermatomyositis (DM) and that DM-associated fasciitis is detectable, as the result of the increased vascularity in the fascia, by power Doppler ultrasonography. We aimed to investigate whether angiogenesis and vascular endothelial growth factor (VEGF)-expressing cells in the fascia are histologically demonstrated during the early phase of DM, and whether inflammation is involved in angiogenesis and an increased number of VEGF-expressing cells.    Methods  We prospectively evaluated 22 patients with DM and 11 patients with polymyositis (PM). Immunohistochemical staining for CD31, VEGF, and tumor necrosis factor-α (TNF-α) were performed on paraffin-embedded sections. The total vascular inflammation score (TVIS), angiogenesis score (AS), and numbers of VEGF-expressing and TNF-α-expressing cells were analyzed in the fascia and muscle.    Results  Significant fasciitis was detected in most of the patients DM with or without myositis-specific/associated antibodies, while mild fasciitis was detected in four patients with PM, two of whom were positive for anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies. The AS and the number of VEGF-expressing cells in the fascia of patients with DM were significantly greater than those of patients with PM; no significant difference was observed in muscle in patients with DM and PM. The number of VEGF-expressing cells in the fascia correlated with the AS of DM patients. In early-phase DM, the AS, the number of VEGF-expressing cells, and the TVIS in the fascia were significantly higher than in muscle. However, no significant differences were observed in these scores excluding the TVIS between muscle and the fascia in late-phase DM. In DM patients, the TVIS correlated with the AS in the fascia, while the number of TNF-α-expressing cells correlated with the TVIS and the number of VEGF-expressing cells in the fascia.    Conclusion  Angiogenesis, the number of VEGF-expressing cells, and the degree of inflammation were higher in the fascia in DM than in PM, and were increased predominantly in the fascia rather than in the muscle in early-phase DM. The degree of inflammation correlated with that of angiogenesis in the fascia of DM. The fascia can therefore be a primary site of inflammation and angiogenesis in the pathogenesis of DM.

Angiogenesis and VEGF-expressing cells are identified predominantly in the fascia rather than in the muscle during the early phase of dermatomyositis