Department of Pediatrics, Mendrisio and Bellinzona, University of Bern, Bern, Switzerland.
Eur J Pediatr. 2011 Dec;170(12):1507-11. doi: 10.1007/s00431-011-1508-4. Epub 2011 Jun 15.
Acute hemorrhagic edema of young children is an uncommon but likely underestimated cutaneous leukocytoclastic vasculitis. The condition typically affects infants 6-24 months of age with a history of recent respiratory illness with or without course of antibiotics. The diagnosis is made in children, mostly nontoxic in appearance, presenting with nonpruritic, large, round, red to purpuric plaques predominantly over the cheeks, ears, and extremities, with relative sparing of the trunk, often with a target-like appearance, and edema of the distal extremities, ears, and face that is mostly non-pitting, indurative, and tender. In boys, the lesions sometimes involve the scrotum and, more rarely, the penis. Fever, typically of low grade, is often present. Involvement of body systems other than skin is uncommon, and spontaneous recovery usually occurs within 6-21 days without sequelae. In this condition, laboratory tests are non-contributory: total blood cell count is often normal, although leukocytosis and thrombocytosis are sometimes found, clotting studies are normal, erythrocyte sedimentation rate and C-reactive protein test are normal or slightly elevated, complement level is normal, autoantibodies are absent, and urinalysis is usually normal. Experienced physicians rapidly consider the possible diagnosis of acute hemorrhagic edema when presented with a nontoxic young child having large targetoid purpuric lesions and indurative swelling, which is non-pitting in character, and make the diagnosis either on the basis of clinical findings alone or supported by a skin biopsy study.
儿童急性出血性水肿是一种罕见但可能被低估的皮肤白细胞碎裂性血管炎。这种疾病通常发生在 6-24 个月大的婴儿身上,有近期呼吸道感染史,伴有或不伴有抗生素治疗史。在外观上,儿童患者通常无中毒症状,主要表现为非瘙痒性、大而圆的红色至紫癜性斑块,主要分布在脸颊、耳朵和四肢,躯干相对较少受累,常呈靶样外观,以及四肢远端、耳朵和面部的水肿,通常是非凹陷性、硬结性和压痛性的。在男孩中,病变有时会累及阴囊,更罕见的情况下会累及阴茎。通常会出现发热,体温通常较低。皮肤以外的其他系统受累并不常见,通常会在 6-21 天内自发恢复,无后遗症。在这种情况下,实验室检查没有帮助:全血细胞计数通常正常,尽管有时会发现白细胞增多和血小板增多,凝血研究正常,红细胞沉降率和 C 反应蛋白试验正常或轻度升高,补体水平正常,自身抗体阴性,尿液分析通常正常。当经验丰富的医生遇到表现为大靶样紫癜性病变和硬结性肿胀、非凹陷性特征、无中毒的年轻患儿时,会迅速考虑急性出血性水肿的可能诊断,并根据临床发现或皮肤活检结果做出诊断。