Food protein-induced enterocolitis syndrome (FPIES) is a type of non-immunoglobulin E (IgE)-mediated food allergy. However, in addition to vomiting and diarrhea, IgE-mediated skin or respiratory symptoms may be comorbidities in some patients with FPIES. We described four unusual cases of neonates with FPIES, whose clinical presentations were variable and misleading. All patients experienced vomiting, diarrhea or other gastrointestinal symptoms, and three of them developed IgE-mediated food allergy. Case 1 was admitted to the hospital with convulsions and then developed severe sepsis and necrotizing enterocolitis (NEC)-like appearance. Case 2 was wrongly diagnosed with Stevens–Johnson syndrome due to a severe extravasation rash of the skin and mucous membranes and a systemic inflammatory response. There was unexplained cholestasis in case 3, which might be attributed to food allergy. Asymptomatic elevation of C-reactive protein was the only hint at early-stage FPIES in case 4. Moreover, there were increased serum food-specific IgG values in three of the above cases. After eliminating the offending food, all of the above clinical manifestations rapidly improved in the four cases; thus, we believe that the most correct diagnosis in the described four cases was FPIES. This case report series should further draw clinicians’ attention to FPIES with variable and atypical symptoms. The usefulness of IgG levels in identifying the presence of FPIES is uncertain.

食物蛋白诱导的小肠结肠炎综合征 (FPIES) 是一种非免疫球蛋白 E (IgE) 介导的食物过敏。然而，除了呕吐和腹泻外，IgE 介导的皮肤或呼吸道症状可能是一些 FPIES 患者的合并症。我们描述了四个不寻常的 FPIES 新生儿病例，其临床表现多变且具有误导性。所有患者均出现呕吐、腹泻或其他胃肠道症状，其中三人出现 IgE 介导的食物过敏。病例 1 因抽搐入院，随后出现严重败血症和坏死性小肠结肠炎 (NEC) 样表现。病例 2 被错误诊断为史蒂文斯-约翰逊综合征，原因是皮肤和黏膜出现严重的外渗性皮疹和全身炎症反应。病例 3 有不明原因的胆汁淤积，这可能归因于食物过敏。在病例 4 中，C 反应蛋白的无症状升高是早期 FPIES 的唯一提示。此外，在上述三个病例中，血清食物特异性 IgG 值升高。4例在排除不良食物后，上述临床表现均迅速好转；因此，我们认为在所描述的四个病例中最正确的诊断是 FPIES。该病例报告系列应进一步引起临床医生对具有多变和非典型症状的 FPIES 的关注。IgG 水平在识别 FPIES 中的作用尚不确定。4例在排除不良食物后，上述临床表现均迅速好转；因此，我们认为在所描述的四个病例中最正确的诊断是 FPIES。该病例报告系列应进一步引起临床医生对具有多变和非典型症状的 FPIES 的关注。IgG 水平在识别 FPIES 中的作用尚不确定。4例在排除不良食物后，上述临床表现均迅速好转；因此，我们认为在所描述的四个病例中最正确的诊断是 FPIES。该病例报告系列应进一步引起临床医生对具有多变和非典型症状的 FPIES 的关注。IgG 水平在识别 FPIES 中的作用尚不确定。