Livedoid vasculitis - リベド血管炎
リベド血管炎 (Livedoid vasculitis) は、主に若年から中年の女性に見られる慢性皮膚疾患です。その特徴を表す頭字語の一つに「下肢に網状パターンを呈し、痛みを伴う紫斑性潰瘍」(PURPLE) があります。慢性静脈高血圧症や静脈瘤など、さまざまな疾患と関連しています。
痛みを伴うこともあります。
relevance score : -100.0%
ReferencesLivedoid vasculopathyは、下肢に再発し続ける痛みを伴う潰瘍が特徴の、まれな皮膚疾患です。
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 は、下肢に再発性の潰瘍を引き起こし、白萎縮として知られる淡い傷跡を残すまれな皮膚疾患です。正確な原因はまだ不明ですが、血液凝固の亢進（hypercoagulability）が主な要因であり、炎症は二次的な役割を果たしていると考えられています。凝固に関与する要因としては、タンパク質C・S欠乏、Factor V Leiden 変異、抗トロンビンIII欠乏、プロトロンビン遺伝子変異、ホモシステイン高値などが挙げられます。生検では、血管内に血栓が形成され、血管壁が肥厚し、瘢痕化が認められます。治療には、抗血小板薬、抗凝固薬、線溶療法など、血栓予防を中心とした多角的アプローチが必要です。この皮膚疾患には、colchicine、hydroxychloroquine、血管拡張薬、免疫抑制薬などが使用されます。
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は、両脚に病変が生じる稀な疾患です。小血管内で血栓が形成される原因は、血管内皮の損傷に加えて、凝固亢進と血栓溶解の低下と考えられています。女性に多く、特に15〜50歳の女性に頻繁に見られます。禁煙や創傷の適切な処置、抗凝固薬や抗血小板薬などの使用が有効です。
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.
