Livedoid vasculitis - Livedoidní Vaskulitida
Livedoidní Vaskulitida (Livedoid vasculitis) je chronické kožní onemocnění, které se vyskytuje převážně u žen v mladém až středním věku. Jedna zkratka používaná k popisu jejích rysů je „Bolestivé purpurové vředy s retikulárním vzorem dolních končetin“ (PURPLE). Je spojena s řadou onemocnění, včetně chronické žilní hypertenze a varixů.
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References
Livedoid vasculopathy je vzácné kožní onemocnění charakterizované bolestivými vředy, které se neustále vracejí na bércích.
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 vzácné kožní onemocnění, které způsobuje opakující se vředy na bércích a zanechávají za sebou bledé jizvy známé jako atrophie blanche. Zatímco přesná příčina je stále nejasná, má se za to, že hlavním faktorem je zvýšená srážlivost krve (hypercoagulability) , přičemž zánět hraje vedlejší roli. Mezi faktory přispívající ke srážení patří následující - deficiencies in proteins C and S, genetic mutations like factor V Leiden, antithrombin III deficiency, prothrombin gene mutations, high levels of homocysteine. Při biopsiích tento stav ukazuje krevní sraženiny uvnitř cév, ztluštění cévních stěn a zjizvení. Léčba zahrnuje mnohostranný přístup zaměřený na prevenci krevních sraženin pomocí léků, jako jsou léky proti krevním destičkám, léky na ředění krve a fibrinolytická terapie. Na toto kožní onemocnění lze použít různé léky - 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 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 vzácný stav, kdy se léze vyskytují na obou nohách. Předpokládá se, že tvorba krevních sraženin v drobných cévách je způsobena zvýšenou srážlivostí a sníženým rozpadem sraženiny spolu s poškozením výstelky cév. Je častější u žen, zejména ve věku 15 až 50 let. Účinné bylo přestat kouřit, ošetřovat rány a užívat léky, jako jsou léky na ředění krve a protidestičkové léky.
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.
