中国麻风皮肤病杂志 ›› 2025, Vol. 41 ›› Issue (9): 689-691.doi: 10.12144/zgmfskin202509689

• 病例报告 • 上一篇    下一篇

儿童嗜酸性肉芽肿性血管炎一例

马春苗1,刘俊枫2,陈军辉1,崔维静1,王建军1   

  1. 1甘肃省人民医院小儿科，甘肃兰州，730000； 2兰州大学第二医院检验医学中心，甘肃兰州，730030
  • 出版日期:2025-09-15 发布日期:2025-09-08

Childhood eosinophilic granulomatous vasculitis: a case report

MA Chunmiao1, LIU Junfeng2, CHEN Junhui1, CUI Weijing1, WANG Jianjun1   

  1. 1 Department of Pediatrics, Gansu Provincial People's Hospital, Lanzhou 730000, China; 2 Laboratory Medicine Center, Lanzhou University Second Hospital, Lanzhou 730030, China
  • Online:2025-09-15 Published:2025-09-08

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摘要： 嗜酸性肉芽肿性血管炎患病率(10.7～13.0)/106，好发于40～60岁，儿童更少见，本文报道一例累及多系统的儿童嗜酸性肉芽肿性血管炎一例。患者，女，13岁，皮损主要表现为丘疹伴出血性水疱、口腔黏膜糜烂，累及的其他系统包括肺部、鼻窦、皮肤、眼部、消化道、肾脏及骨髓。皮肤组织病理示：基底层细胞液化变性并表皮下疱形成，真皮部分血管弹力纤维及毛囊纤维素样坏死，间质水肿伴密集的淋巴细胞浸润。胞浆型抗中性粒细胞胞浆抗体 (c-ANCA) 1∶32阳性。骨髓穿刺检查：粒系有成熟障碍及感染现象，嗜酸性粒细胞明显增多，占40%左右。肺部浸润严重，进展为呼吸衰竭。予大剂量甲泼尼龙冲击、丙种球蛋白及环磷酰胺治疗后好转。

关键词: 嗜酸性肉芽肿性血管炎, 皮疹, 坏疽, 儿童

Abstract: The prevalence of eosinophilic granulomatosis with polyangiitis (EGPA) is (10.7-13.0)/106. It predominantly occurs in people aged 40-60 years, and is even rarer in children. We reports a case of childhood eosinophilic granulomatosis with polyangiitis involving multiple systems.The patient was a 13-year-old girl. The main manifestations of skin lesions were papules accompanied by hemorrhagic blisters and oral mucosal erosion. Other involved systems included the lungs, paranasal sinuses, skin, eyes, digestive tract, kidneys and bone marrow. Skin biopsy showed liquefaction degeneration of basal cells with subepidermal blister formation, fibrinoid necrosis of elastic fibers in some dermal blood vessels and hair follicles, interstitial edema with dense lymphocytic infiltration. Cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) was positive at a titer of 1∶32. Bone marrow aspiration examination revealed maturation disorder and infection signs in the granulocytic series. Eosinophils were significantly increased, accounting for about 40%. Pulmonary infiltration was severe, progressing to respiratory failure. The patient improved after treatment with high-dose methylprednisolone pulse therapy, intravenous immunoglobulin and cyclophosphamide.

Key words: eosinophilic granulomatosis with polyangiities, skin rash, gangrene, children