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首页> 外文期刊>American Journal of Dermatopathology >Histological Classification and Differential Diagnosis of Nonepisodic Angioedema With Eosinophilia: A Clinicopathologic Study of 12 Cases With Literature Review
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Histological Classification and Differential Diagnosis of Nonepisodic Angioedema With Eosinophilia: A Clinicopathologic Study of 12 Cases With Literature Review

机译：非间歇性血管性水肿伴嗜酸性粒细胞增多的组织学分类和鉴别诊断：附文献复习的12例临床病理研究

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摘要

Nonepisodic angioedema with eosinophilia (NEAE) is a rare condition characterized with monoepisodic angioedema, a nonfebrile state, eosinophilia, normal serum IgM levels, and lack of internal organ involvement. The histology of this disease is not yet well known. The purpose of this study was to characterize the histopathologic features of NEAE. Twelve cases of clinically confirmed NEAE were retrieved from 6 institutions, and these cases were reviewed regarding the clinical data and histopathology, particularly regarding granulomatous lesions. The authors demonstrated that the histology of NEAE can be classified into 3 patterns that of eosinophilic granulomatous panniculitis (7/12 cases), eosinophilic dermatitis without granuloma formation (3/12 cases), and invisible dermatosis (2/12 cases). Six of the 7 granulomatous cases showed the characteristic eosinophilic granulomatous lesions containing individual necrotic adipocytes with membranous fat changes, which could be a differential clue to the diagnosis of NEAE. Review of the previously reported cases (n = 37) revealed that the histological classification could be adaptable to these reported cases. The authors should recognize the histological variation of NEAE and distinguish it from the histological mimickers, including eosinophilic granulomatosis with polyangiitis, erythema nodosum, hypereosinophilic syndrome, and episodic angioedema with eosinophilia.
机译：非间歇性血管性水肿伴嗜酸性粒细胞增多症（NEAE）是一种罕见病，其特征为单发性血管性水肿，非发热状态，嗜酸性粒细胞增多，血清IgM水平正常和缺乏内脏器官。这种疾病的组织学尚不清楚。这项研究的目的是表征NEAE的组织病理学特征。从6个机构中检索了12例经临床证实的NEAE病例，并对这些病例进行了临床数据和组织病理学检查，尤其是肉芽肿病变。作者证明，NEAE的组织学可分为嗜酸性肉芽肿性脂膜炎（7/12例），无肉芽肿形成的嗜酸性皮肤炎（3/12例）和不可见皮肤病（2/12例）三种模式。 7例肉芽肿病例中有6例表现出特征性嗜酸性肉芽肿病变，其中含有单个坏死的脂肪细胞，具有膜性脂肪变化，这可能是诊断NEAE的不同线索。回顾先前报告的病例（n = 37），发现组织学分类可能适用于这些报告的病例。作者应认识到NEAE的组织学变异，并将其与组织学上的模仿者区分开，包括嗜酸性肉芽肿合并多血管炎，结节性红斑，嗜酸性粒细胞增多综合征和发作性血管性水肿伴嗜酸性粒细胞增多。

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