Livedoid vasculitis - Livedoidni Vaskulitis
Livedoidni Vaskulitis (Livedoid vasculitis) je kronična kožna bolest koja se javlja pretežno kod žena u mlađim i srednjim godinama. Jedan akronim koji se koristi za opisivanje njegovih karakteristika je „Bolni purpurni ulkusi s retikularnim uzorkom na donjim ekstremitetima“ (PURPLE). Povezana je s nizom bolesti, uključujući kroničnu vensku hipertenziju i varikozitet.
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ReferencesLivedoid vasculopathy je rijetko stanje kože koje karakterišu bolni čirevi koji se stalno vraćaju na potkoljenice.
Livedoid vasculopathy (LV) is a rare thrombotic vasculopathy of the dermis characterized by painful, relapsing ulcers over the lower extremities. Diagnosis is challenging due to the overlap in clinical appearance and nomenclature with other skin disorders. Treatment selection is complicated by poor understanding of the pathogenesis of LV and lack of robust clinical trials evaluating therapy efficacy. The terminology and pathophysiology of LV are reviewed here, along with its epidemiology, clinical and histologic features, and treatment options. A diagnostic pathway is suggested to guide providers in evaluating for comorbidities, referring to appropriate specialists, and choosing from the available classes of therapy.
Livedoid vasculopathy (LV) is a rare thrombotic vasculopathy of the dermis characterized by painful, relapsing ulcers over the lower extremities. Diagnosis is challenging due to the overlap in clinical appearance and nomenclature with other skin disorders. Treatment selection is complicated by poor understanding of the pathogenesis of LV and lack of robust clinical trials evaluating therapy efficacy. The terminology and pathophysiology of LV are reviewed here, along with its epidemiology, clinical and histologic features, and treatment options. A diagnostic pathway is suggested to guide providers in evaluating for comorbidities, referring to appropriate specialists, and choosing from the available classes of therapy.
Livedoid vasculopathy je rijetko stanje kože koje uzrokuje ponavljajuće čireve na potkoljenicama, ostavljajući iza sebe blijede ožiljke poznate kao atrofija blanš. Iako je tačan uzrok još uvijek nejasan, smatra se da je povećano zgrušavanje krvi (hypercoagulability) glavni faktor, dok upala ima sporednu ulogu. Faktori koji doprinose zgrušavanju uključuju: nedostatak proteina C i S (deficiencies in proteins C and S), genetske mutacije poput faktora V Leiden (factor V Leiden), nedostatak antitrombina III (antithrombin III deficiency), mutacije gena protrombina (prothrombin gene mutations) i povišene nivoe homocisteina (high levels of homocysteine). U biopsijama se vide trombi unutar krvnih žila, zadebljanje zidova žila i ožiljci. Liječenje zahtijeva višestruki pristup usmjeren na prevenciju tromboze lijekovima poput antitrombocitnih agenata, antikoagulansa i fibrinolitičke terapije. Za ovo kožno stanje mogu se koristiti i drugi lijekovi – kolhicin (colchicine) i hidroksiklorokin (hydroxychloroquine), vazodilatatori i imunosupresivi.
Livedoid vasculopathy is a rare skin condition that causes recurring ulcers on the lower legs, leaving behind pale scars known as atrophie blanche. While the exact cause is still unclear, it's believed that increased blood clotting (hypercoagulability) is the main factor, with inflammation playing a secondary role. Factors contributing to clotting include as follows - deficiencies in proteins C and S, genetic mutations like factor V Leiden, antithrombin III deficiency, prothrombin gene mutations, high levels of homocysteine. In biopsies, the condition shows blood clots inside vessels, thickening of vessel walls, and scarring. Treatment involves a multi-pronged approach focusing on preventing blood clots with medications like anti-platelet drugs, blood thinners, and fibrinolytic therapy. Various drugs can be used for this skin condition - colchicine and hydroxychloroquine, vasodilators, immunosuppressants.
Livedoid vasculopathy is a rare skin condition that causes recurring ulcers on the lower legs, leaving behind pale scars known as atrophie blanche. While the exact cause is still unclear, it's believed that increased blood clotting (hypercoagulability) is the main factor, with inflammation playing a secondary role. Factors contributing to clotting include as follows - deficiencies in proteins C and S, genetic mutations like factor V Leiden, antithrombin III deficiency, prothrombin gene mutations, high levels of homocysteine. In biopsies, the condition shows blood clots inside vessels, thickening of vessel walls, and scarring. Treatment involves a multi-pronged approach focusing on preventing blood clots with medications like anti-platelet drugs, blood thinners, and fibrinolytic therapy. Various drugs can be used for this skin condition - colchicine and hydroxychloroquine, vasodilators, immunosuppressants.
Livedoid vasculopathy je rijetko stanje u kojem se lezije javljaju na obje noge. Smatra se da je stvaranje krvnih ugrušaka u sićušnim krvnim žilama uzrokovano povećanim zgrušavanjem i smanjenom razgradnjom ugrušaka, uz oštećenje sluznice krvnih žila. Bolest je češća kod žena, najčešće u dobi od 15 do 50 godina. Prestanak pušenja, pravilna njega rana i upotreba lijekova, poput anticoagulants (razrjeđivači krvi) i antiplatelet drugs (lijekovi protiv trombocita), bili su učinkoviti.
Livedoid vasculopathy is a rare vasculopathy that is typically characterized by bilateral lower limb lesions. Increased thrombotic activity and decreased fibrinolytic activity along with endothelial damage are believed to be the cause of thrombus formation in the capillary vasculature. It is 3 times more common in females than in males, especially in patients between the ages of 15 to 50 years. There is no definite first-line treatment, but general measures like smoking cessation, wound care, and pharmacological measures like anticoagulants and antiplatelets have shown good results.
Livedoid vasculopathy is a rare vasculopathy that is typically characterized by bilateral lower limb lesions. Increased thrombotic activity and decreased fibrinolytic activity along with endothelial damage are believed to be the cause of thrombus formation in the capillary vasculature. It is 3 times more common in females than in males, especially in patients between the ages of 15 to 50 years. There is no definite first-line treatment, but general measures like smoking cessation, wound care, and pharmacological measures like anticoagulants and antiplatelets have shown good results.
