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大疱性类天疱疮样皮疹伴嗜酸性粒细胞增多综合征。

Bullous pemphigoid-like rash revealing hypereosinophilic syndrome.

机构信息

Department of Dermatology, Mohammed V University of Rabat, Rabat, Morocco

Department of Dermatology, Mohammed V University of Rabat, Rabat, Morocco.

出版信息

BMJ Case Rep. 2021 Jun 8;14(6):e242695. doi: 10.1136/bcr-2021-242695.

Abstract

Hypereosinophilic syndrome (HES) is a rare haematologic disorder characterised by unexplained, persistent eosinophilia with organ involvement. We report the case of a 74-year-old patient who presented with a pemphigoid-like bullous rash as the only manifestation of an HES. This report highlights the importance of the dermatological manifestations of this potentially life-threating condition that may initially present as a skin-limited disease. Bullous pemphigoid (BP) is a very common autoimmune blistering dermatosis that may share common clinical and histological features or associate with an HES. HES should be considered when patients diagnosed with BP demonstrate atypical course under corticosteroids and/or recalcitrant blood eosinophilia.

摘要

嗜酸性粒细胞增多综合征(HES)是一种罕见的血液系统疾病,其特征为不明原因的持续性嗜酸性粒细胞增多,并伴有器官受累。我们报告了一例 74 岁患者,其唯一表现为 HES 的是类天疱疮样大疱性皮疹。本报告强调了这种潜在危及生命的疾病的皮肤科表现的重要性,其最初可能表现为皮肤局限性疾病。大疱性类天疱疮(BP)是一种非常常见的自身免疫性水疱性皮肤病,可能具有共同的临床和组织学特征,或与 HES 相关。当诊断为 BP 的患者在皮质类固醇治疗下表现出非典型病程和/或难治性血嗜酸性粒细胞增多时,应考虑 HES。

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Pemphigus foliaceus progressing to bullous pemphigoid.落叶型天疱疮进展为大疱性类天疱疮。
J Dermatol. 2019 Nov;46(11):e424-e426. doi: 10.1111/1346-8138.15021. Epub 2019 Jul 25.
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Biologic Agents for the Treatment of Hypereosinophilic Syndromes.治疗嗜酸性粒细胞增多综合征的生物制剂。
J Allergy Clin Immunol Pract. 2017 Nov-Dec;5(6):1502-1509. doi: 10.1016/j.jaip.2017.08.001.

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