Abstract：
The purpose of the study is to highlight clinical signs that are either suggestive of or against the diagnosis of AHEI to improve diagnosis and management. The medical records of children under 3 years old diagnosed with AHEI were retrospectively reviewed. Clinical data and photographs were reviewed by three independent experts, and the cases were classified as probable, doubtful, or unclear AHEI. Of the 69 cases of children diagnosed with AHEI included in 22 centers, 40 were classified as probable, 22 as doubtful, and 7 as unclear. The median age of patients with probable AHEI was 11 months [IQR 9-15], and they were in overall good condition (n = 33/40, 82.5%). The morphology of the purpura was targetoid in 75% of cases (n = 30/40) and ecchymotic in 70% of cases (n = 28/40) and affected mostly the legs (n = 39/40, 97%), the arms (n = 34/40, 85%), and the face (n = 33/40, 82.5%). Edema was observed in 95% of cases and affected mostly the hands (n = 36/38, 95%) and feet (n = 28/38, 74%). Pruritus was absent in all patients with probable AHEI and described for 6/21 with doubtful AHEI (29%). AHEI was the original diagnosis in only 24 patients (n = 24/40, 60%). The major differential diagnoses were purpura fulminans and urticaria multiforme. Conclusion: AHEI, which the diagnosis is made on clinical findings, is often misdiagnosed. Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in a young child with a good overall condition are highly suggestive of AHEI. What is Known: •Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis affecting children under 3 years old. •Appropriate diagnosis is important to distinguish this benign disease from more serious diseases to avoid investigations and treatments, iatrogenic harm and unnecessary follow-up. What is New: •AHEI is an uncommon disorder often misdiagnosed by pediatricians and dermatologists. •Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in an infant with a good overall condition are highly suggestive of AHEI.
摘要：
该研究的目的是突出提示或反对AHEI诊断的临床体征，以改善诊断和管理。回顾性分析了3岁以下被诊断为AHEI的儿童的医疗记录。临床数据和照片由三名独立专家审查，这些病例被归类为可能的，令人怀疑,或不清楚AHEI。在22个中心的69例诊断为AHEI的儿童中，40人被归类为可能，22令人怀疑，7不清楚。可能患有AHEI的患者的中位年龄为11个月[IQR9-15]，总体状况良好(n=33/40，82.5%)。紫癜的形态在75％的病例中（n=30/40）是目标样的，在70％的病例中（n=28/40）是瘀斑，并且主要影响腿部（n=39/40，97％）。臂(n=34/40，85%)，和面部（n=33/40，82.5%）。在95％的病例中观察到水肿，并且主要影响手（n=36/38，95％）和脚（n=28/38，74％）。所有可能的AHEI患者均无瘙痒，并描述为6/21的可疑AHEI患者（29％）。AHEI是仅24例患者的原始诊断（n=24/40，60％）。主要鉴别诊断为暴发性紫癜和多形性荨麻疹。结论:AHEI,根据临床发现做出诊断，经常被误诊。位于面部/耳朵的紫癜性病变，手臂/前臂，在整体状况良好的幼儿中，大腿/腿部有手部水肿而无瘙痒，高度提示AHEI。已知:•婴儿期急性出血性水肿(AHEI)是影响3岁以下儿童的皮肤白细胞碎裂性血管炎。•适当的诊断对于区分这种良性疾病和更严重的疾病是很重要的，以避免调查和治疗。医源性伤害和不必要的后续行动。新增内容：•AHEI是一种罕见的疾病，经常被儿科医生和皮肤科医生误诊。•位于面部/耳朵上的紫癜性病变,手臂/前臂，在整体状况良好的婴儿中，大腿/腿部有手部水肿而无瘙痒，高度提示AHEI。
