Livedoid vasculitis - Vasculite Livedoide
La Vasculite Livedoide (Livedoid vasculitis) è una patologia cutanea cronica, più frequente nelle donne giovani e di mezza età. Un acronimo utilizzato per descriverne le caratteristiche è “Ulcere purpuriche dolorose a disegno reticolare degli arti inferiori” (VIOLA). È associata a numerose patologie, tra cui l’ipertensione venosa cronica e le varicosità.
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ReferencesLivedoid vasculopathy è una rara condizione cutanea caratterizzata da ulcere dolorose ricorrenti nella parte inferiore delle gambe.
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 è una rara condizione della pelle che provoca ulcere ricorrenti nella parte inferiore delle gambe, lasciando cicatrici pallide note come atrofia bianca. Sebbene la causa esatta non sia ancora chiara, si ritiene che il fattore principale sia l’aumento della coagulazione del sangue (hypercoagulability), mentre l’infiammazione svolge un ruolo secondario. I fattori che contribuiscono alla coagulazione includono: carenze delle proteine C e S, mutazioni genetiche come il fattore V Leiden, carenza di antitrombina III, mutazioni del gene della protrombina e livelli elevati di omocisteina. Nelle biopsie la condizione mostra coaguli di sangue all’interno dei vasi, ispessimento delle pareti vascolari e cicatrici. Il trattamento prevede un approccio multimodale incentrato sulla prevenzione della formazione di coaguli, mediante farmaci antipiastrinici, anticoagulanti e terapia fibrinolitica. Diversi farmaci possono essere impiegati per questa patologia cutanea: colchicine e hydroxychloroquine, vasodilatatori, immunosoppressori.
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 è una condizione rara in cui le lesioni compaiono su entrambe le gambe. Si ritiene che la formazione di coaguli di sangue nei minuscoli vasi sia dovuta a un aumento della coagulazione e a una ridotta fibrinolisi, insieme a danni al rivestimento vascolare. È più comune nelle donne, soprattutto tra i 15 e i 50 anni. Smettere di fumare, prendersi cura delle ferite e utilizzare farmaci come anticoagulanti e antipiastrinici si sono rivelati efficaci.
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.
