表现为紫癜及大疱的嗜酸性肉芽肿性多血管炎一例

Eosinophilic granulomatosis with polyangiitis presented as purpura and bullae: a case report

嗜酸性肉芽肿性多血管炎是一种自身免疫性疾病,特征表现为成人期发病的哮喘、外周血及组织嗜酸粒细胞增多及小血管炎。本文报道一例,患者有哮喘及慢性鼻窦炎病史,外周血嗜酸粒细胞明显增多,皮肤组织病理示:真皮全层嗜酸粒细胞浸润,坏死性小血管炎及血管外肉芽肿,基底膜带及真皮浅中层血管壁C3颗粒状沉积。给予泼尼松25 mg/d及外用卤米松治疗,皮疹逐渐消退。

Eosinophilic Granulomatosis with Polyangiitis(EGPA)is an autoimmune disease characterized by adult-onset asthma,peripheral blood and tissue eosinophilia,and microvasculitis.We report a patient with a history of asthma and chronic sinusitis,a significant increase in peripheral blood eosinophils,and histopathologic findings of dermal eosinophile infiltration,necrotizing small vasculitis,and extravascular granuloma,granular deposition of C3 in the basement membrane zone and vascular wall of the superficial and middle dermis.The therapy of oral prednisone(25 mg/day)and topical halometasone cream was given,and the rash and bulla gradually subsided.