ATYPICAL FOOD PROTEIN-INDUCED ENTEROCOLITIS SYNDROME (FPIES) TO SHELLFISH IN A PEDIATRIC PATIENT

Abstract

<h3>Introduction</h3> Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated food allergy syndrome. Ingestion of food protein causes delayed onset of repetitive projectile vomiting, pallor, diarrhea, lethargy 1-4 hours after intake. Diagnosis is clinical. Pathophysiology involves antigen presentation and TNFα alteration of the intestinal tight junction barrier resulting in fluid shifts and symptoms. Atypical FPIES is rare and includes concomitant IgE sensitization to the food trigger, potentially from ‘Th2-skewing'. The majority of FPIES to seafood are published for adolescents/adults. Our case describes a rare presentation of atypical FPIES to shrimp in infancy. <h3>Case Description</h3> Patient is an 8-year-old male with PMH eczema who presented for evaluation. As a 12-month-old, the patient developed 3 episodes of projectile vomiting and mild lethargy 3 hours after shrimp ingestion. No associated IgE-mediated symptoms. He went to the urgent care and improved with zofran. Family history included atopic conditions of allergic rhinitis and eczema. Testing demonstrated positive skin prick testing (SPT) to shrimp (3 mm wheal) and positive shrimp-IgE (1.49 kU/L). Most recently, he had a repeat reaction to shellfish at age 8 with 4 episodes of vomiting and paleness 2-3 hours after ingestion. <h3>Discussion</h3> Up to 20% of FPIES cases may transform over time to an IgE-mediated phenotype. These atypical FPIES cases have been shown to have longer persistence, as seen in our patient (average tolerance to solid food triggers is age 4). Epinephrine should be prescribed in atypical FPIES due to the risk of anaphylaxis. Treatment includes food avoidance. Further work is essential to determine a disease biomarker.