Tense blisters and hemorrhagic bullae as the first manifestation of eosinophilic granulomatosis with polyangiitis.

IF 0.9 Q4 RHEUMATOLOGY
Hajime Inokuchi, Mitsuhiro Akiyama, Hiroto Horikawa, Yasushi Kondo, Shuntaro Saito, Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko
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引用次数: 0

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) poses a significant diagnostic challenge due to its varied clinical presentation. Here, we present a case of a 59-year-old female with a history of asthma and sinusitis, who manifested with an extremely rare presentation of drastic tense blisters and hemorrhagic bullae alongside purpuric lesions and peripheral neuropathy. Examinations revealed eosinophilia, positive anti-neutrophil cytoplasmic antibody, and characteristic pathological findings with small vessel vasculitis in the purpura. Treatment with glucocorticoids and cyclophosphamide led to rapid improvement in peripheral eosinophilia, skin manifestations and motor neuron deficits. Although rare, our case underscores that bullous skin lesions should be recognized as a potential cutaneous hallmark of EGPA to aid timely diagnosis, since prompt treatment initiation is crucial given the potential irreversible organ damage and poor prognosis of EGPA.

嗜酸性粒细胞肉芽肿伴多血管炎的首发症状--张力性水疱和出血性大疱。
嗜酸性粒细胞肉芽肿伴多血管炎(EGPA)的临床表现多种多样,给诊断带来了巨大挑战。这里,我们介绍了一例 59 岁的女性患者,她有哮喘和鼻窦炎病史,表现为极罕见的剧烈紧张性水疱和出血性大疱,同时伴有紫癜性病变和周围神经病变。检查发现患者嗜酸性粒细胞增多,抗中性粒细胞胞浆抗体阳性,病理结果显示紫癜中伴有小血管炎。使用糖皮质激素和环磷酰胺治疗后,外周嗜酸性粒细胞增多、皮肤表现和运动神经元功能障碍迅速改善。尽管罕见,但我们的病例强调,应将大疱性皮肤损害视为 EGPA 的潜在皮肤特征,以帮助及时诊断,因为鉴于 EGPA 可能造成不可逆的器官损伤且预后不良,及时开始治疗至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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CiteScore
1.40
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