BMJ Case Reports 2018; doi:10.1136/bcr-2017-222822
  • Rare disease

Food protein-induced enterocolitis syndrome: a challenging diagnosis

  1. Isabel Pinto Pais3
  1. 1Department of Paediatrics, Hospital Center of Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
  2. 2Pediatric Gastroenterology, Centro Hospitalar Gaia Espinho, Vila Nova de Gaia, Portugal
  3. 3Pediatrics Department, CHGE, Porto, Portugal
  1. Correspondence to Dr Andreia Ribeiro, andreia.ribeiru{at}
  • Accepted 26 January 2018
  • Published 8 February 2018


Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated gastrointestinal food hypersensitivity triggered by food proteins. It may present acutely, with repetitive vomiting, diarrhoea and lethargy leading to dehydration and eventually shock or insidiously with intermittent emesis, chronic diarrhoea or failure to thrive. We describe a paediatric male patient with recurrent sepsis-like episodes of fever, lethargy, ashen-grey skin colouration and vomiting followed by diarrhoea. These episodes were triggered by cow’s milk formula and grains. Laboratory tests revealed leucocytosis, thrombocytosis, metabolic acidosis and elevated C reactive protein. After exclusion of other differential diagnoses, the diagnosis of FPIES was established on clinical improvement with withdrawal of the offending food and positive oral food challenge. FPIES diagnosis requires a high index of suspicion and is frequently delayed, which contributes to an increased morbidity. This is due to the wide spectrum of clinical presentations and due to the absence of specific diagnostic tests.


  • Contributors All named authors have contributed to this work. The manuscript has been read and approved by all named authors. Specifically, AR has contributed in literature research, acquisition and data analysis, conception and writing the article. DM has contributed in literature research, acquisition and data analysis, and drafting the article. CC and IPP have participated in conception, writing and revision of the article for important scientific content.

  • Competing interests None declared.

  • Patient consent Guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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