嗜酸性肉芽肿性多血管炎(EGPA),以前叫Churg-Strauss综合征,是一种罕见的慢性坏死性嗜酸性肉芽肿性炎性疾病,其特征是富含嗜酸性粒细胞的肉芽肿性炎症和与支气管哮喘和嗜酸性粒细胞增多相关的中小型血管血管炎,在大约50-70%的病例中,抗中性粒细胞胞浆抗体(ANCA)呈阳性。我们报告了一例23岁的妇女因双下肢小囊泡和双下肢小散斑皮疹伴瘙痒V6的4个月病史而入院,4个月有散斑皮疹伴瘙痒。外周血的实验室数据显示白细胞增多,嗜酸性粒细胞增多,血小板增多症,纤溶亢进,和轻度肾损伤。她的ANCA是阴性的,皮肤病理检查显示肉芽肿病变伴嗜酸性粒细胞,而骨髓中也发现了嗜酸性粒细胞升高。EGPA被诊断。另一方面,患者有2年的鼻-鼻窦炎,长达9个月的肾病综合征,长达一个月的干咳,这可能是一种哮喘。使用类固醇治疗,然后进行全身免疫调节治疗,病人的症状缓解了。我们的病例报告和文献综述强调了将咳嗽变异性哮喘作为EGPA的初始症状的重要性。提供早期诊断和治疗的机会,以降低疾病进一步进展和发病率的风险。
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a rare chronic necrotizing eosinophilic granulomatous inflammatory disease characterized by eosinophil-rich granulomatous inflammation and small- to medium-size vessel vasculitis associated with bronchial asthma and eosinophilia, which is positive for anti-neutrophil cytoplasmic antibody (ANCA) in approximately 50-70% of cases. We report a
case of a 23-year-old woman was admitted to our hospital because of a of small vesicles on both lower limbs and a 4-month history of small scattered skin rash with pruritus V6 on both lower limbs four-month history of scattered skin rash with pruritus. Laboratory data from peripheral blood revealed leukocytosis, eosinophilia, thrombocytosis, hyperfibrinolysis, and mild renal injury. Her ANCA was negative, and the skin pathological examination showed granuloma lesions with eosinophils, while elevated eosinophils were also found in the bone marrow. EGPA was diagnosed. On the other hand, the patient had 2-year-long rhinosinusitis, 9-month-long nephrotic syndrome, and 1-month-long dry cough, which might be a type of asthma. With steroid therapy followed by systemic immunomodulatory therapy, the patient\'s symptoms were relieved. Our
case report and literature review highlight the importance of recognizing cough variant asthma as an initial presenting symptom of EGPA, providing an opportunity for early diagnosis and treatment to reduce the risk of further disease progression and morbidity.