Abstract：
Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil-cytoplasmic-antibody (ANCA)-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation. Symptoms of skin involvement can appear in 30-50% of patients with GPA, and may present as the initial presentation. Case Presentation: We describe two patients who presented with multiple deep, large, nonhealing skin ulcers postoperatively with purulent drainage and fever. Both patients were diagnosed with GPA after an extensive evaluation, including histopathology. Infectious, connective tissue disease and malignant etiologies were excluded. Their cANCA and PR3-ANCA levels were positive. Patient 2 was diagnosed early and recovered well after treatment with corticosteroids and rituximab; however, Patient 1 had a poor prognosis due to a long disease course. Conclusions: Diseases with multiple deep, large skin ulcers and fever can be infectious or noninfectious. Atypical manifestations may lead to missed diagnosis and misdiagnosis. GPA may initially present in a localized form before progressing to a generalized disease. The two cases we have highlighted will prompt clinicians to nevertheless call for a low threshold for diagnosis.
摘要：
背景：肉芽肿性多血管炎（GPA）是一种抗中性粒细胞胞浆抗体（ANCA）相关的小血管血管炎，其特征是坏死性肉芽肿性炎症。30-50％的GPA患者可出现皮肤受累的症状，并可能作为初始演示文稿出现。病例介绍：我们描述了两名患者，他们表现出多个深，大,术后皮肤溃疡不愈合，脓性引流和发热。经过广泛评估，两名患者均被诊断为GPA，包括组织病理学.传染性，排除结缔组织疾病和恶性病因。他们的cANCA和PR3-ANCA水平为阳性。患者2被早期诊断，在使用皮质类固醇和利妥昔单抗治疗后恢复良好；然而，患者1由于病程较长而预后不良。结论：多种深部疾病，大面积皮肤溃疡和发热可为感染性或非感染性。不典型表现可能导致漏诊和误诊。GPA最初可以在发展为全身性疾病之前以局部形式存在。我们强调的两个病例将促使临床医生呼吁低诊断阈值。
