Pearls and pitfalls in food protein-induced enterocolitis syndrome (FPIES)

BACKGROUND: Food protein–induced enterocolitis syndrome (FPIES) is a rare, non–immunoglobulin E (IgE) mediated gastrointestinal food hypersensitivity.

It is a clinical diagnosis commonly characterized by profuse vomiting 1 to 4 hours after ingestion of the triggering food(s).

OBJECTIVE: The objective was to increase awareness of FPIES and review the epidemiology, clinical presentation, pathogenesis, diagnosis, and management of FPIES.

The lack of availability of a definite biomarker or diagnostic tool often leads to a delay in diagnosis.

METHODS: A literature search of salient articles that described case reports and case series of FPIES and their management were analyzed.

RESULTS: A case of FPIES with a literature review is presented with emphasis on clinical pearls and pitfalls.

FPIES is a diagnosis of exclusion and the mainstay of treatment is avoidance of the trigger food(s) for at least 12–18 months from the last exposure.

CONCLUSION: As FPIES is a non-IgE-mediated reaction, allergy testing via skin-prick test or blood tests to measure food IgE antibodies is not routinely recommended.

Many children outgrow FPIES by 3–4 years of age.

Supervised oral food challenge is recommended to assess acquisition of tolerance.