Taylor Braunberger MD , Jessica S. Mounessa BS , Ryan O'Leary MD , Ekama Carlson MD, PhD , Sabrina Newman MD
{"title":"嗜酸性肉芽肿病合并多血管炎患者的下肢威胁动脉血栓形成","authors":"Taylor Braunberger MD , Jessica S. Mounessa BS , Ryan O'Leary MD , Ekama Carlson MD, PhD , Sabrina Newman MD","doi":"10.1016/j.jccw.2017.03.001","DOIUrl":null,"url":null,"abstract":"<div><p><span><span>Eosinophilic granulomatosis<span> (EGPA), or Churg-Strauss syndrome, is a rare and necrotizing systemic vasculitis<span>, which affects small-to-medium-sized vessels and often manifests with severe asthma and </span></span></span>eosinophilia<span><span><span>. We report a case of a 72 year-old male with a two-year lung-biopsy proven history of EGPA who presented with retiform purpura and patchy necrosis on his bilateral shins, which progressed to sharply demarcated, stellate </span>ulcerations<span><span> with surrounding erythema within two weeks. Laboratory work up revealed elevated anti-Cardiolipin IgM, rheumatoid factor, </span>erythrocyte sedimentation rate, and C-reactive protein, although P-neutrophil cytoplasmic antibody (P-ANCA) and C-neutrophil cytoplasmic antibody (C-ANCA) were negative. Vascular studies revealed long anterior tibial and dorsalis pedis </span></span>artery occlusion and severe small vessel disease in plantar </span></span>digital arteries<span><span>. Despite treatment with intravenous </span>cyclophosphamide<span><span>, pulse-dose methylprednisolone<span>, and pentoxifylline, the patient experienced </span></span>disease progression<span><span> and limb threatening arterial thrombosis. This case highlights the importance of vascular and neuropathic </span>sequelae that may result from untreated or undertreated EGPA in P-ANCA-negative patients without active pulmonary symptoms.</span></span></span></p></div>","PeriodicalId":90358,"journal":{"name":"The journal of the American College of Clinical Wound Specialists","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2016-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jccw.2017.03.001","citationCount":"3","resultStr":"{\"title\":\"Limb-Threatening Arterial Thrombosis in a Patient with Eosinophilic Granulomatosis with Polyangiitis\",\"authors\":\"Taylor Braunberger MD , Jessica S. Mounessa BS , Ryan O'Leary MD , Ekama Carlson MD, PhD , Sabrina Newman MD\",\"doi\":\"10.1016/j.jccw.2017.03.001\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p><span><span>Eosinophilic granulomatosis<span> (EGPA), or Churg-Strauss syndrome, is a rare and necrotizing systemic vasculitis<span>, which affects small-to-medium-sized vessels and often manifests with severe asthma and </span></span></span>eosinophilia<span><span><span>. We report a case of a 72 year-old male with a two-year lung-biopsy proven history of EGPA who presented with retiform purpura and patchy necrosis on his bilateral shins, which progressed to sharply demarcated, stellate </span>ulcerations<span><span> with surrounding erythema within two weeks. Laboratory work up revealed elevated anti-Cardiolipin IgM, rheumatoid factor, </span>erythrocyte sedimentation rate, and C-reactive protein, although P-neutrophil cytoplasmic antibody (P-ANCA) and C-neutrophil cytoplasmic antibody (C-ANCA) were negative. Vascular studies revealed long anterior tibial and dorsalis pedis </span></span>artery occlusion and severe small vessel disease in plantar </span></span>digital arteries<span><span>. Despite treatment with intravenous </span>cyclophosphamide<span><span>, pulse-dose methylprednisolone<span>, and pentoxifylline, the patient experienced </span></span>disease progression<span><span> and limb threatening arterial thrombosis. This case highlights the importance of vascular and neuropathic </span>sequelae that may result from untreated or undertreated EGPA in P-ANCA-negative patients without active pulmonary symptoms.</span></span></span></p></div>\",\"PeriodicalId\":90358,\"journal\":{\"name\":\"The journal of the American College of Clinical Wound Specialists\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2016-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1016/j.jccw.2017.03.001\",\"citationCount\":\"3\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"The journal of the American College of Clinical Wound Specialists\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2213510317300040\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"The journal of the American College of Clinical Wound Specialists","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2213510317300040","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Limb-Threatening Arterial Thrombosis in a Patient with Eosinophilic Granulomatosis with Polyangiitis
Eosinophilic granulomatosis (EGPA), or Churg-Strauss syndrome, is a rare and necrotizing systemic vasculitis, which affects small-to-medium-sized vessels and often manifests with severe asthma and eosinophilia. We report a case of a 72 year-old male with a two-year lung-biopsy proven history of EGPA who presented with retiform purpura and patchy necrosis on his bilateral shins, which progressed to sharply demarcated, stellate ulcerations with surrounding erythema within two weeks. Laboratory work up revealed elevated anti-Cardiolipin IgM, rheumatoid factor, erythrocyte sedimentation rate, and C-reactive protein, although P-neutrophil cytoplasmic antibody (P-ANCA) and C-neutrophil cytoplasmic antibody (C-ANCA) were negative. Vascular studies revealed long anterior tibial and dorsalis pedis artery occlusion and severe small vessel disease in plantar digital arteries. Despite treatment with intravenous cyclophosphamide, pulse-dose methylprednisolone, and pentoxifylline, the patient experienced disease progression and limb threatening arterial thrombosis. This case highlights the importance of vascular and neuropathic sequelae that may result from untreated or undertreated EGPA in P-ANCA-negative patients without active pulmonary symptoms.