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