MESOGLYCAN TREATMENT RESTORES DEFECTIVE FIBRINOLYTIC POTENTIAL IN CUTANEOUS NECROTIZING VENULITIS

Background. Cutaneous necrotizing venulitis (CNV) is a clinical disord er associated with segmental inflammation and fibrinoid necrosis of th e dermal venules. It usually presents clinically as palpable purpura, even sometimes as nodules, bullae, ulcers, and urticarial lesions. Thi s form, when showing as leukocytoclastic vasculitis is apparently char acterized by the tissue deposition of circulating immune complexes and by reduced cutaneous (CFA) and plasma (PFA) fibrinolytic activity due to reduced release of plasminogen activator (PA) from the venular end otheliocytes. Reduced CFA and PFA cause large amounts of fibrin deposi ts in both intra- and perivascular areas, which are able to magnify an d self perpetuate the inflammatory processes following immune complex deposition. Methods. We have studied both the PFA and CFA potential (t he maximum amount of PA released in the skin after certain stimuli) an d the deposits of immunoglobulins, C3, and fibrin related antigen, bef ore and after intradermal injection of histamine (a substance able to provoke endothelial release Of PA), in three subjects affected by CNV before and 20 days after 10 mg/kg/day I.M. treatment with the fibrinol ytic agent mesoglycan. Results. Cutaneous fibrinolytic activity and CF A potential, reduced prior to treatment, was normal after treatment, w hile the deposits of immunoglobulins (IgA, IgG and IgM), C3, and fibri n related antigen, detected with direct immune fluorescence (DIF) show ed similar findings before and after treatment. Conclusions. These dat a suggest that reduced CFA may play a major role in the pathogenesis o f the immunologically mediated injury in CNV. The intraperivascular de position of fibrin is favored. The fibrinolytic agent mesoglycan seems effective in restoring defective fibrinolysis in patients affected by cutaneous necrotizing venulitis, suggesting that in cases with reduce d cutaneous fibrinolitic activity (or potential) the use of a fibrinol ytic agent should be considered.