肠病性肢端皮炎：临床表现与儿科诊断
ACRODERMATITIS ENTEROPATHICA: CLINICAL MANIFESTATIONS AND PEDIATRIC DIAGNOSIS.
作者信息
Ciampo Ieda Regina Lopes Del, Sawamura Regina, Ciampo Luiz Antonio Del, Fernandes Maria Inez Machado
机构信息
Universidade Federal de São Carlos, São Carlos, São Paulo, Brasil.
Universidade de São Paulo, Ribeirão Preto, São Paulo, Brasil.
出版信息
Rev Paul Pediatr. 2018 Apr-Jun;36(2):238-241. doi: 10.1590/1984-0462/;2018;36;2;00010. Epub 2018 Jan 15.
OBJECTIVE
To report a case of acrodermatitis enteropathica, a rare disease with autosomal recessive inheritance.
CASE DESCRIPTION
An 11-month-old boy was presenting symmetrical erythematous and yellowish-brownish crusted lesions on his face, feet, hands and knees, intermittent diarrhea, fever, and recurrent infections since the age of six months. He was thin and had scarce hair on the scalp. The serum zinc level was measured and a reduced level of 27.0 mcg/dL (normal range: 50.0-120.0) was identified. Oral supplementation with 2.0 mg/kg/day of zinc sulfate was immediately initiated. A rapid and progressive improvement of symptoms was observed. The symptoms reappeared with an attempt to stop supplementation.
COMMENTS
Recognizing and properly treating acrodermatitis enteropathica is important to prevent complications.
目的
报告一例肠病性肢端皮炎病例，这是一种罕见的常染色体隐性遗传病。
病例描述
一名11个月大的男孩自6个月大以来，面部、足部、手部和膝盖出现对称性红斑及黄棕色结痂性皮损，伴有间歇性腹泻、发热和反复感染。他体型消瘦，头皮毛发稀少。检测血清锌水平，发现其降低至27.0微克/分升（正常范围：50.0 - 120.0）。立即开始口服硫酸锌，剂量为2.0毫克/千克/天。观察到症状迅速且逐渐改善。试图停止补充锌时，症状再次出现。
评论
认识并正确治疗肠病性肢端皮炎对于预防并发症很重要。
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