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VASCULITIS AND VASCULOPATHY

Kod ulceracija donjih ekstremiteta, najvažniju ulogu, među mnogobrojnim poznatim sastavnicama patofiziološkog procesa, imaju oštećenja krvnih žila. Vaskulitisom se označava heterogena skupina kliničkih entiteta, kojima je zajedničko obilježje upalni proces stijenke arterija i vena bilo koje veličine i u bilo kojem organu, a u koži vrlo često. Kod vaskulopatija riječ je o oštećenju stijenki krvnih žila i kapilara, npr. nekim medikamentima. Klasifikacija vaskulitisa prema veličini krvne žile služi za razumijevanje među kliničarima i istraživačima, a ne kao dijagnostičko sredstvo. Prema histološkom nalazu, pregledom bioptata stijenke krvne žile, vaskulitisi se mogu podijeliti u tri skupine: limfocitni, leukocitoklastični i anulomatozni. livedoidni vaskulitis (“livedo retikularis”) najčešče pogađa žene i lokaliziran je uglavnom na donjim ekstremitetima. Etiologija liveidnog vaskulitisa može biti posljedica autoimunih bolesti, posljedica opstrukcije kapilara krioglobulinima ili antifosfolipoidnog sindroma. livedoidna vaskulopatija (lV) je hijalinizacijska bolest vaskulature, s trombozama i ulceracijama na donjim ekstremitetima, nepoznate etiologije. lV je izdvojena kao zasebna bolest koja obično nije posljedica drugih primarnih bolesti.obilježja lV su: 71% oboljelih su žene, prosječna životna dob je 45 godina, raspon je 10-85 godina, bolest u 80,8% slučajeva zahvaća donje ekstremitete bilateralno, u 68,9% slučajeva bolest se prezentira ulceracijama, iza ulceracija može se razviti atrophie blanche u 71,1% slučajeva, u 74,1% slučajeva nalazi se smanjenje u transkutanoj oksimetriji, u 22,2% bolesnika nalazi se mutacija u faktoru V (heterozigoti leiden), smanjena je aktivnost proteina C u 13,3% slučajeva, mutacija gena za protrombin (G20210A) u 8,3% slučajeva, lupus antikoagulant pozitivan u 17,9% bolesnika, antikardiolipinska antitijela pozitivna u 28,6% bolesnika, povišena je razina homocisteina u 14,3% bolesnika, histološki pregled krvne žile pokazuje intaluminalnu trombozu u 97,8% bolesnika, direktna imunofluorescencija uzorka krvne žile pokazuje imunoglobuline i komponente komplementa u krvnim žilama na površini, u sredini dermisa ali i duboko u dermisu. Imunoflouorescentna slika različita je od bolesti imunih kompleksa. neki od lijekova kojima se pokušava liječiti lV su: pentoksifilin niskomolekularni heparin, hiperbarična oksigenoterapija, metilprednizolon i.v. s pentoksifilinom, rekombinantni tkivni aktivator plazminogena, intravenski imunoglobulini, kombinacija fenformina (bigvanid) i etilestrenola (anabolički steroid), varfarin, heparin, sistemska fotokemoterapija (PuVA terapija s peroralnim uzimanjem psoralena), niskomolekularni dekstran. inficirane ulceracije liječe se antibioticima. Može se primijeniti kombinirana terapija folnom kiselinom, vitaminom B12 i vitaminom B6.Many pathophysiological process components are known to be implicated in lower limb ulcerations, among which vascular lesions have a major role. Vasculitis denotes a heterogeneous group of clinical entities which all are characterized by the inflammatory process of arterial and venous walls of any size and in any organ, quite frequently in the skin. Vasculopathy, on the other hand, refers to vascular and capillary lesions caused by, for example, some medications. The classification of vasculitides according to the size of the Blood Vessels involved serves for proper understanding the issue among clinicians and researchers, and not as a diagnostic tool. According to histologic finding obtained by examination of Blood Vessel Biopsy specimen, vasculitides are divided into three groups: lymphocytic, leukocytoclastic and granulomatous. Livedoid vasculitis (livedo reticularis) most commonly affects women and is generally localized on lower extremities. The etiology of livedoid vasculitis may imply autoimmune diseases, capillary obstruction with cryoglobulins, or antiphospholipid syndrome. Livedoid vasculopathy is a hyalinization disease of the vasculature, with thromboses and ulcerations on lower extremities, and of unknown etiology. Livedoid vasculopathy has been singled out as a separate disease that usually does not occur consequentially to other primary diseases. Livedoid vasculopathy typically affects women (71%) at a mean age of 45 (range 10-85) years; bilateral involvement of both lower limbs is present in 80.8%, disease manifested with ulcerations in 68.9%, ulcerations followed by development of atrophie blanche in 71.1%, transcutaneous oximetry reduction is found in 74.1%, factor V mutation (Leiden heterozygotes) in 22.2%, reduced protein C activity in 13.3%, prothrombin gene mutation (G20210A) in 8.3%, positive lupus anticoagulant in 17.9%, positive anticardiolipin antibodies in 28.6%, and elevated homocysteine level in 14.3% cases; Blood Vessel histology shows intraluminal thrombosis in 97.8% of patients, while direct immunofluorescence of Blood Vessel specimen shows immunoglobulins and complement components in Blood Vessels on the surface, in the mid-dermis as well as deep in the dermis. The immunofluorescence pattern differs from that found in immune complex diseases. Some of the agents tried in the treatment of livedoid vasculopathy include pentoxifylline, low-molecular heparin, hyperbaric oxygen therapy, methylprednisolone i.v. with pentoxifylline, recombinant tissue plasminogen activator, intravenous immunoglobulins, phenformin (biguanide) and ethylestrenol (anabolic steroid) combination, warfarin, heparin, systemic photochemotherapy (PUVA with oral psoralen), and low-molecular dextran. Infected ulcerations are treated with antibiotics. Combined therapy with folic acid, vitamin B12 and vitamin B6 can also be used