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婴儿急性出血性水肿:普通儿科医生的罕见诊断。

Acute Hemorrhagic Edema of Infancy: an unusual diagnosis for the general pediatrician.

作者信息

Cunha Diego Fontana Siqueira, Darcie Ana Letícia Fornazieri, Benevides Gabriel Nuncio, Ferronato Angela Espósito, Hein Noely, Lo Denise Swei, Yoshioka Cristina Ryoka Miyao, Hirose Maki, Cardoso Debora Morais, Gilio Alfredo Elias

机构信息

Department of Pediatrics - Hospital das Clínicas - Faculty of Medicine - Universidade de São Paulo, São Paulo/SP - Brazil .

Faculty of Medicine - Universidade de São Paulo, São Paulo/SP - Brazil .

出版信息

Autops Case Rep. 2015 Sep 30;5(3):37-41. doi: 10.4322/acr.2015.020. eCollection 2015 Jul-Sep.

Abstract

Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis, clinically characterized by the classical triad: palpable purpuric skin lesions, edema and fever, and is commonly misdiagnosed as Henoch-Schönlein purpura. In addition to its sudden onset, AHEI is also characterized by its self-limited course with complete and spontaneous recovery occurring between 1 and 3 weeks. Because of the scarcity of studies on therapy with corticosteroids, the conservative approach is usually recommended. The authors report an unusual case of an one-year-old boy who presented with typical cutaneous rash of AHEI and orchitis, the latter showing complete resolution after less than 24 hours of prednisolone therapy. The authors call attention to this entity mainly as a differential diagnosis of Henoch-Schönlein purpura and to the importance of new studies to establish the benefits of corticosteroid therapy for AHEI.

摘要

婴儿急性出血性水肿(AHEI)是一种罕见的白细胞破碎性血管炎,临床特征为典型的三联征:可触及的紫癜性皮肤病变、水肿和发热,常被误诊为过敏性紫癜。除了起病突然外,AHEI还具有自限性病程的特点,在1至3周内可完全自发恢复。由于关于皮质类固醇治疗的研究较少,通常建议采用保守方法。作者报告了一例不寻常的病例,一名一岁男孩出现了AHEI典型的皮肤皮疹和睾丸炎,后者在泼尼松龙治疗不到24小时后完全消退。作者主要提醒注意这一疾病,作为过敏性紫癜的鉴别诊断,并强调开展新研究以确定皮质类固醇治疗AHEI的益处的重要性。

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