无汗性外胚层发育不良(AED)是一种罕见的遗传性疾病,其特征为来源于外胚层的结构缺乏或数量减少。临床表现为无汗症、毛发稀少、无牙或有少量畸形牙。由于无汗症,因此在感染性疾病中,高热可能是致命性的并发症。近年有2篇报道强调了AED成人患者的鼻部并发症。作者报告1例年仅4个月的男婴,因首先出现令人厌恶的鼻腔分泌物而后诊断AED的病例。病例男婴,4个月时其父母发现婴儿的双侧鼻腔有持续性分泌物并结痂,皮肤苍白,但眼周皮肤色黑伴皱纹。8个月时因肺部感染入院治疗。根据患儿呼吸道感染,皮肤苍白生长迟缓等症状,考虑为囊 Anaerobic ectodermal dysplasia (AED) is a rare genetic disorder characterized by a lack of structure or reduced number of structures derived from ectoderm. Clinical manifestations of sweat-free, rare hair, no teeth or a small amount of deformed teeth. Due to anhidrosis, high fever may be a fatal complication in infectious diseases. In recent years, two articles have highlighted nasal complications in AED adult patients. The authors report that one male patient, who was only 4 months old, was diagnosed with AEDs by first showing disgusting nasal secretions. Case of baby boy, 4 months old parents found that the baby’s bilateral nasal secretions and persistent discharge scab, pale skin, but dark brown skin with eye wrinkles. 8 months due to pulmonary infection admitted to hospital. According to children with respiratory infections, pale and slow growth of the skin and other symptoms, consider the capsule