Braunberger Taylor, Mounessa Jessica S, O'Leary Ryan, Carlson Ekama, Newman Sabrina
University of North Dakota, Fargo, ND, USA.
University of Colorado Hospital, Aurora, CO, USA.
J Am Coll Clin Wound Spec. 2017 Mar 27;8(1-3):28-30. doi: 10.1016/j.jccw.2017.03.001. eCollection 2016.
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.
嗜酸性肉芽肿性多血管炎（EGPA），即变应性肉芽肿性血管炎，是一种罕见的坏死性系统性血管炎，累及中小血管，常表现为重度哮喘和嗜酸性粒细胞增多。我们报告一例72岁男性，有经肺活检证实的两年EGPA病史，其双下肢胫前出现网状紫癜和片状坏死，两周内进展为边界清晰的星状溃疡并伴有周围红斑。实验室检查显示抗心磷脂IgM、类风湿因子、红细胞沉降率和C反应蛋白升高，尽管抗蛋白酶3中性粒细胞胞浆抗体（P-ANCA）和抗髓过氧化物酶中性粒细胞胞浆抗体（C-ANCA）均为阴性。血管检查显示胫前动脉和足背动脉长段闭塞，足底趾动脉存在严重的小血管病变。尽管给予静脉环磷酰胺、冲击剂量甲泼尼龙和己酮可可碱治疗，患者仍出现疾病进展和威胁肢体的动脉血栓形成。该病例强调了在无活动性肺部症状的P-ANCA阴性患者中，未经治疗或治疗不足的EGPA可能导致的血管和神经病变后遗症的重要性。
