Thrombotic vasculopathy, immunothrombosis, and endotheliopathy in iMCD-TAFRO. (A) Acral skin with full thickness epidermal necrosis (original magnification, ×100). (B) Small vessels in the dermis and subcutaneous fat show thrombi (arrowhead; original magnification, ×200). (C) iMCD-TAFRO, inflammation, and immunothrombosis. (D) ELISA for FVIIa/AT, FVIIa/antithrombin complex. (E) MP-TF functional assay (TF activity was blocked by the inhibitor Ixolaris²²). ELISA for citrullinated histones, a marker for neutrophil extracellular traps (NETs) (F), soluble endothelial protein C receptor (sEPCR) (G), thrombomodulin (H), and PAI-1 (I). Array 2 shows the fold change in coagulation factors (J) and components of the complement cascade (K). Array 1 shows plasma levels for E-selectin (L), sICAM-1 (M), sICAM-3 (N), sVCAM-1 (O), Tie-2 (P), bFGF (Q), and sFlt1 (R). (S) Array 2 shows the fold change in markers associated with endothelial cell activation and angiogenesis. Array 1 and ELISA results are for controls (Controls), flare (Flare), 4 days after treatment (Treatment Day 4), and in remission (Remission) 6 months later. Array 2 results are expressed as fold change during the flare vs remission.