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The pattern observed in the SLE cohort was clearly distinct from NP and MMD with regard to vascular density, vascular damage, and neoangiogenesis. CONCLUSIONS MMD is not associated with microvascular changes of the nailfold capillaries. In this respect, it is clearly distinct from SLE. © 2020 The Authors. Microcirculation published by John Wiley Sons Ltd.Heterozygous mutations in the Berardinelli-Seip congenital lipodystrophy 2 (BSCL2) gene have been reported with different clinical phenotypes including Silver syndrome (SS)/spast